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Sample records for huntington county indiana

  1. Quarry Quest. A Field Trip Guide to the Indiana Limestone District, Monroe and Lawrence Counties, Indiana.

    Science.gov (United States)

    Shewmaker, Sherman N.

    This guide provides information for planning a field trip to the Indiana Limestone District. This district, located in Monroe and Lawrence Counties, Indiana, is responsible for material that has dominated the building-limestone market in the United States for nearly a century. A few of the many well-known buildings using Indiana limestone are the…

  2. Water resources of Indiana County, Pennsylvania

    Science.gov (United States)

    Williams, D.R.; McElroy, T.A.

    1997-01-01

    Indiana County, west-central Pennsylvania, is a major producer of coal and natural gas. Water managers and residents are concerned about the effects of mining and natural gas exploration on the surface- and ground-water resources of the county. This study assesses the quality and quantity of water in Indiana County. Ground- and surface-water sources are used for public supplies that serve 61 percent of the total population of the county. The remaining 39 percent of the population live in rural areas and rely on cisterns and wells and springs that tap shallow aquifers. Most of the county is underlain by rocks of Middle to Upper Pennsylvanian age. From oldest to youngest, they are the Allegheny Group, the Glenshaw Formation, the Casselman Formation, and the Monongahela Group. Almost all the coals mined are in the Allegheny Group and the Monongahela Group. Ground water in Indiana County flows through fractures in the rock. The size and extent of the fractures, which are controlled by lithology, topography, and structure, determine the sustained yield of wells. Topography has a significant control over the yields of wells sited in the Allegheny Group. Properly sited wells in the Glenshaw Formation may have yields adequate for municipal, commercial, or industrial uses. The Casselman Formation yields adequate amounts of water for domestic use. Yield of the Monongahela Group is small, and the water may not be of suitable quality for most uses. Yields of hilltop wells may be marginal, but valley wells may yield sufficient amounts for large-volume users. Data on the other rock units are sparse to nonexistent. Few wells in the county yield more than 40 gallons per minute. Most of the wells that do are in valleys where alluvial deposits are extensive enough to be mapable. Short-term water-level fluctuations are variable from well to well. Seasonal water-level fluctuations are controlled by time of year and amount of precipitation. The quality of water from the Casselman

  3. Parcels and Land Ownership, Published in 2011, Huntington County Government.

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — This Parcels and Land Ownership dataset as of 2011. The extent of these data is generally Huntington County, IN. This metadata was auto-generated through the Ramona...

  4. Indiana Underground Railroad Folklore: Western Route and Daviess County.

    Science.gov (United States)

    Shelton, Lois G.

    Materials for teaching a unit about the Underground Railroad (the system set up to assist fleeing, runaway slaves heading north) in Indiana are presented. Specifically, the Western Route that passed through Daviess County in Indiana is examined. The materials provide background on the Underground Railroad and the Western Route, plans for teaching…

  5. Road and Street Centerlines - COUNTY_STREET_CENTERLINES_IDHS_IN: Street Centerlines Maintained by County Agencies in Indiana (Indiana Department of Homeland Security, Line Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — COUNTY_STREET_CENTERLINES_IDHS_IN is a line shapefile that contains street centerlines maintained by county agencies in Indiana, provided by personnel of Indiana...

  6. Road and Street Centerlines - COUNTY_STREET_CENTERLINES_IDHS_IN: Street Centerlines Maintained by County Agencies in Indiana (Indiana Department of Homeland Security, Line Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — COUNTY_STREET_CENTERLINES_IDHS_IN is a line feature class that contains street centerlines maintained by county agencies in Indiana, provided by personnel of Indiana...

  7. Address Points - COUNTY_ADDRESS_POINTS_IDHS_IN: Address Points Maintained by County Agencies in Indiana (Indiana Department of Homeland Security, Point Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — COUNTY_ADDRESS_POINTS_IDHS_IN is a point shapefile that contains address points maintained by county agencies in Indiana, provided by personnel of Indiana Department...

  8. Road and Street Centerlines - COUNTY_STREET_CENTERLINES_IDHS_IN: Street Centerlines Maintained by County Agencies in Indiana (Indiana Department of Homeland Security, Line Shapefile)

    Data.gov (United States)

    NSGIC State | GIS Inventory — COUNTY_STREET_CENTERLINES_IDHS_IN is a line feature class that contains street centerlines maintained by county agencies in Indiana, provided by personnel of Indiana...

  9. County and Parish Boundaries - COUNTY_GOVERNMENT_BOUNDARIES_IDHS_IN: Governmental Boundaries Maintained by County Agencies in Indiana (Indiana Department of Homeland Security, Polygon feature class)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — COUNTY_GOVERNMENT_BOUNDARIES_IDHS_IN is a polygon feature class that contains governmental boundaries maintained by county agencies in Indiana, provided by personnel...

  10. County and Parish Boundaries - COUNTY_GOVERNMENT_BOUNDARIES_IDHS_IN: Governmental Boundaries Maintained by County Agencies in Indiana (Indiana Department of Homeland Security, Polygon feature class)

    Data.gov (United States)

    NSGIC State | GIS Inventory — COUNTY_GOVERNMENT_BOUNDARIES_IDHS_IN is a polygon feature class that contains governmental boundaries maintained by county agencies in Indiana, provided by personnel...

  11. Government Districts, Other - COUNTY_GOVERNMENT_BOUNDARIES_IDHS_IN: Governmental Boundaries Maintained by County Agencies in Indiana (Indiana Department of Homeland Security, Polygon Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — COUNTY_GOVERNMENT_BOUNDARIES_IDHS_IN is a polygon shapefile that contains governmental boundaries maintained by county agencies in Indiana, provided by personnel of...

  12. Address Points - COUNTY_ADDRESS_POINTS_IDHS_IN: Address Points Maintained by County Agencies in Indiana (Indiana Department of Homeland Security, Point feature class)

    Data.gov (United States)

    NSGIC State | GIS Inventory — COUNTY_ADDRESS_POINTS_IDHS_IN is an ESRI Geodatabase point feature class that contains address points maintained by county agencies in Indiana, provided by personnel...

  13. 78 FR 28503 - Approval and Promulgation of Air Quality Implementation Plans; Indiana; Lake and Porter Counties...

    Science.gov (United States)

    2013-05-15

    ... Porter Counties, Indiana, 1997 8-Hour Ozone Maintenance Plan and 1997 Annual Fine Particulate Matter... Porter Counties State Implementation Plans (SIPs) for the 1997 8-hour ozone standard, and the 1997 annual... MOVES2010a-based budgets for the Lake and Porter County, Indiana 1997 8-hour ozone maintenance area and the...

  14. OrthoImagery submittal for Huntington County, Indiana

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — Digital orthographic imagery datasets contain georeferenced images of the Earth's surface, collected by a sensor in which object displacement has been removed for...

  15. 2011-2013 Indiana Statewide Imagery and LiDAR Program: Lake Michigan Watershed Counties

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — Indiana's Statewide LiDAR data is produced at 1.5-meter average post spacing for all 92 Indiana Counties covering more than 36,420 square miles. New LiDAR data was...

  16. 78 FR 28550 - Approval and Promulgation of Air Quality Implementation Plans; Indiana; Lake and Porter Counties...

    Science.gov (United States)

    2013-05-15

    ... Porter Counties, Indiana, 1997 8-Hour Ozone Maintenance Plan and 1997 Annual Fine Particulate Matter... and Porter State Implementation Plans (SIPs) for the 1997 8-hour ozone standard, and the 1997 annual...

  17. The Overmyer mastodon (Mammut americanum) from Fulton County, Indiana

    Science.gov (United States)

    Woodman, N.; Branstrator, J.W.

    2008-01-01

    In June 1978 the partial skeleton of an American mastodon, Mammut americanum, was salvaged from a drainage ditch in Fulton County, north-central Indiana. The remains were recovered mostly from ca. 170-260 cm below the current land surface in marl overlain by peat and peaty marl. The stratigraphy of the site indicates that the remains were deposited in a small, open-water pond that subsequently filled. The skeleton, which is 41-48% complete, is that of a mature female, ca. 30-34 y old at death based on dental eruption and wear. Postcranial bone measurements indicate that this individual was relatively large for a female. Radiocarbon dating of wood from under the pelvis of the mastodon provided a maximum date of 12,575 ? 260 14C y BP [15,550-13,850 cal y BP] for the animal, which is up to 2575 14C y before the species is believed to have become extinct. Pollen samples from the site corroborate the interpretation that the regional climate was cooler and more humid than at present and supported a mixed spruce-deciduous parkland assemblage. The relatively small size of the molars of this and other mastodons from Indiana supports the hypothesis that late-glacial mastodons - just prior to their extinction - were smaller in size relative to earlier, full-glacial conspecifics. The relationship between molar size and body size is not clear, however, and there may be geographical factors as well as a temporal influence to size variation in these animals.

  18. Demographic Data - Census_County_TIGER00_IN: Indiana Counties Census Data 2000 (Derived from U.S. Census Bureau, 1:24,000 Polygon Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — Census_County_TIGER00_IN contains locations for all Indiana counties and contains census data for each county. Data is from U.S. Department of Commerce, U.S. Census...

  19. Earlier sediment differential compaction in Gibson County, Indiana

    Science.gov (United States)

    Eggert, D.L.

    1987-01-01

    A thick wedge of nonmarine gray shale extends from the Galatia Channel and overlies the Springfield Coal Member of the Petersburg Formation (Pennsylvania) in western Gibson Country, but mainly marine black shales overlie the Springfield in the eastern part of the county. The sulfur content of the Springfield coal is lower beneath this wedge of nonmarine gray shale than beneath the marine shales. Fine-grained, compacted bay and overbank deposits form the western delta platform of the coal. Less compacted deposits of fluvial channel sandstone make up the eastern delta platform. Differential compaction of the delta platform of the Springfield coal controlled gray-shale distribution and determined the sulfur content of the coal. ?? 1987.

  20. Agricultural Chemicals in Leary Weber Ditch Basin, Hancock County, Indiana, 2003-04

    Science.gov (United States)

    Baker, Nancy T.; Lathrop, Timothy R.

    2006-01-01

    Leary Weber Ditch Basin, Hancock County, Indiana, is part of an Agricultural Chemicals: Source, Transport, and Fate study conducted by the National Water-Quality Assessment Program of the U.S. Geological Survey. Water-quality samples were collected in Leary Weber Ditch and in the major hydrologic compartments of the Leary Weber Ditch Basin during 2003 and 2004. Hydrologic compartments that contribute water and agricultural chemicals to Leary Weber Ditch are rain water, overland-flow water, soil water, tile-drain water, and ground water. Samples were analyzed for selected pesticides, nutrients, and major ions.

  1. Streamflow and water quality of the Grand Calumet River, Lake County, Indiana, and Cook County, Illinois, October 1984

    Energy Technology Data Exchange (ETDEWEB)

    Crawford, C.G.; Wangsness, D.J.

    1987-01-01

    A diel water-quality survey was done to investigate the sources of dry-weather waste inputs attributable to other than permitted point-source effluent and to evaluate the waste-load assimilative capacity of the Grand Calumet River, Lake County, Indiana, and Cook County, Illinois, in October 1984. Flow in the Grand Calumet River consists almost entirely of municipal and industrial effluents which comprised more than 90% of the 500 cu ft/sec flow observed at the confluence of the East Branch Grand Calumet River and the Indiana Harbor Ship Canal during the study. At the time of the study, virtually all of the flow in the West Branch Grand Calumet River was municipal effluent. Diel variations in streamflow of as much as 300 cu ft/sec were observed in the East Branch near the ship canal. The diel variation diminished at the upstream sampling sites in the East Branch. In the West Branch, the diel variation in flow was quite drastic. Average dissolved-oxygen concentrations at stations in the East Branch ranged from 5.7 to 8.2 mg/L and at stations in the West Branch from 0.8 to 6.6 mg/L. Concentrations of dissolved solids, suspended solids, biochemical-oxygen demand, ammonia, nitrite, nitrate, and phosphorus were substantially higher in the West Branch than in the East Branch. In the East Branch, only the Indiana Stream Pollution Control Board water-quality standards for total phosphorus and phenol were exceeded. In the West Branch, water-quality standards for total ammonia, chloride, cyanide, dissolved solids, fluoride, total phosphorus, mercury, and phenol were exceeded and dissolved oxygen was less than the minimum allowable. 25 refs., 65 figs., 15 tabs.

  2. Mercury in Precipitation in Indiana, January 2001-December 2003

    Science.gov (United States)

    Risch, Martin R.

    2007-01-01

    Mercury in precipitation was monitored during 2001 through 2003 at four locations in Indiana as part of the National Atmospheric Deposition Program-Mercury Deposition Network (NADP-MDN). Monitoring stations were operated at Roush Lake near Huntington, Clifty Falls State Park near Madison, Monroe County Regional Airport near Bloomington, and Indiana Dunes National Lakeshore near Porter. At these monitoring stations, precipitation amounts were measured and weekly samples were collected for analysis of total mercury and methylmercury by low-level methods. Wet deposition was computed with the total mercury and methylmercury concentrations and the precipitation amounts.

  3. Streamflow and water quality of the Grand Calumet River, Lake County, Indiana, and Cook County, Illinois, October 1984

    Science.gov (United States)

    Crawford, Charles G.; Wangsness, David J.

    1987-01-01

    A diel (24-hour) water-quality survey was done to investigate the sources of dry-weather waste inputs attributable to other than permitted point-source effluent and to evaluate the waste-load assimilative capacity of the Grand Calumet River, Lake County, Indiana, and Cook County, Illinois, in October 1984. Flow in the Grand Calumet River consists almost entirely of municipal and industrial effluents which comprised more than 90% of the 500 cu ft/sec flow observed at the confluence of the East Branch Grand Calumet River and the Indiana Harbor Ship Canal during the study. At the time of the study, virtually all of the flow in the West Branch Grand Calumet River was municipal effluent. Diel variations in streamflow of as much as 300 cu ft/sec were observed in the East Branch near the ship canal. The diel variation diminished at the upstream sampling sites in the East Branch. In the West Branch, the diel variation in flow was quite drastic; complete reversals of flow were observed at sampling stations near the ship canal. Average dissolved-oxygen concentrations at stations in the East Branch ranged from 5.7 to 8.2 mg/L and at stations in the West Branch from 0.8 to 6.6 mg/L. Concentrations of dissolved solids, suspended solids, biochemical-oxygen demand, ammonia, nitrite, nitrate, and phosphorus were substantially higher in the West Branch than in the East Branch. In the East Branch, only the Indiana Stream Pollution Control Board water-quality standards for total phosphorus and phenol were exceeded. In the West Branch, water-quality standards for total ammonia, chloride, cyanide, dissolved solids, fluoride, total phosphorus, mercury, and phenol were exceeded and dissolved oxygen was less than the minimum allowable. Three areas of significant differences between cumulative effluent and instream chemical-mass discharges were identified in the East Branch and one in the West Branch. The presence of unidentified waste inputs in the East Branch were indicated by

  4. DIGITAL FLOOD INSURANCE RATE MAP DATABASE, HUNTINGTON COUNTY, INDIANA (AND INCORPORATED AREAS)

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — The Digital Flood Insurance Rate Map (DFIRM) Database depicts flood risk information and supporting data used to develop the risk data. The primary risk...

  5. Dietary Behaviors Associated With Fruit and Vegetable Consumption, Marion County, Indiana, 2005

    Directory of Open Access Journals (Sweden)

    Karl W. Staser

    2011-05-01

    Full Text Available IntroductionEating inadequate amounts of fruits and vegetables is associated with diminished health, and most Americans fall short of the Centers for Disease Control and Prevention’s recommendation to eat at least 2 servings of fruit and 3 servings of vegetables each day. This study assessed behaviors associated with fruit and vegetable consumption in adults.MethodsA cross-sectional, random-digit–dialed telephone survey of 4,784 adults living in Marion County (Indianapolis, Indiana, measured demographic characteristics, personal health data, food consumption, food label use, and other eating habits. Multivariate logistic regressions were used to assess the association between selected dietary behaviors and fruit and vegetable consumption, controlling for demographic characteristics.ResultsBehaviors associated with adequate versus inadequate consumption of fruits and vegetables were frequent snacking on healthy foods (odds ratio [OR], 2.54, eating meals at home (OR, 2.09, using nutrition labels when making purchases (OR, 1.52, and using “heart healthy” symbols and other food information labels when ordering from restaurants (OR, 1.41. Frequent red meat consumption was negatively associated with adequate consumption of fruits and vegetables (OR, 0.64.ConclusionsHealthful snacking, food label use, and eating meals prepared at home may improve dietary quality. Our measure of adequacy may also be useful in future studies assessing dietary behavior and diet composition.

  6. Landscape consequences of natural gas extraction in Armstrong and Indiana Counties, Pennsylvania, 2004–2010

    Science.gov (United States)

    Slonecker, Terry E.; Milheim, Lesley E.; Roig-Silva, Coral M.; Malizia, Alexander R.

    2013-01-01

    Increased demands for cleaner burning energy, coupled with the relatively recent technological advances in accessing unconventional hydrocarbon-rich geologic formations, have led to an intense effort to find and extract natural gas from various underground sources around the country. One of these sources, the Marcellus Shale, located in the Allegheny Plateau, is currently undergoing extensive drilling and production. The technology used to extract gas in the Marcellus Shale is known as hydraulic fracturing and has garnered much attention because of its use of large amounts of fresh water, its use of proprietary fluids for the hydraulic-fracturing process, its potential to release contaminants into the environment, and its potential effect on water resources. Nonetheless, development of natural gas extraction wells in the Marcellus Shale is only part of the overall natural gas story in this area of Pennsylvania. Conventional natural gas wells are commonly located in the same general area as the Marcellus Shale and are frequently developed in clusters across the landscape. The combined effects of these two natural gas extraction methods create potentially serious patterns of disturbance on the landscape. This document quantifies the landscape changes and consequences of natural gas extraction for Armstrong County and Indiana County in Pennsylvania between 2004 and 2010. Patterns of landscape disturbance related to natural gas extraction activities were collected and digitized using National Agriculture Imagery Program (NAIP) imagery for 2004, 2005/2006, 2008, and 2010. The disturbance patterns were then used to measure changes in land cover and land use using the National Land Cover Database (NLCD) of 2001. A series of landscape metrics is also used to quantify these changes and is included in this publication.

  7. Preliminary water-quality assessment of the upper White River near Indianapolis, Marion County, Indiana

    Science.gov (United States)

    Wangsness, David J.; Eikenberry, S.E.; Wilber, W.G.; Crawford, Charles G.

    1981-01-01

    The White River Park Commission is planning the development of park facilities along the White River through Indianapolis, Ind. A key element in the planning is the determination of whether water quality of the river is suitable for recreation. A preliminary water-quality assessment conducted August 4-5, 1980, indicated that, during low-flow steady-state conditions, the river is suitable for partial body contact recreation (any contact with water up to, but not including complete submergence). Dissolved-oxygen concentrations varied but were higher than the Indiana water-quality standards established to ensure conditions for the maintenance of a well-balanced, warm-water fish community. High fecal-coliform densities that have been observed in the White River during high streamflow are probably caused by stormwater runoff carried by combined storm and sanitary sewers. However, during the low-flow, steady-state conditions on August 4-5, 1980, fecal-coliform densities were within the Indiana standards for partial body contact recreation. Quantities of organic matter and concentrations of nutrients and heavy metals in the White River were generally within the limits recommended by the U.S. Environmental Protection Agency and were generally similar to values for other Indiana rivers. Chromium, copper, lead, zinc, and mercury are accumulating in bottom materials downstream from 30th Street. The phytoplankton concentrations in the White River were high. The dominant phytoplankton species were indicative of rivers moderately affected by organic wastes. (USGS)

  8. Geochemical and γ ray characterization of Pennsylvanian black shales: Implications for elevated home radon levels in Vanderburgh County, Indiana.

    Science.gov (United States)

    Scheller, Kent W; Elliott, William S

    2015-10-01

    Radon ((222)Rn) is a radioactive gas that results from the decay of uranium ((238)U) in the Earth's crust. This study characterizes the presence and relative quantity of radon precursors in the Pennsylvanian black shales of southwest Indiana. Cores were drilled on the campus of the University of Southern Indiana to a depth of 237.7 m (780 ft) during exploration for coal-bed methane. Gamma ray logs were taken to measure radioactive activity as a function of depth in the bore hole. Activity readings of 270, 467, 555, and 388 GAPI (American Petroleum Institute γ ray units) were measured at depths of 124.3 m (408 ft), 154.0 m (505 ft), 187.1 m (614 ft) and 214.0 m (702 ft) in four separate shale layers of the Pennsylvanian stratigraphic column. GAPI units are used in the petroleum industry when drilling to represent the relative intensities of γ radiation from (40)K, (232)Th, and (238)U in bore holes (Belknap et al., 1959). For purposes of this study, the high activity readings on the gamma ray logs were used only to identify at which depths further gamma ray spectroscopy of the cores would be completed in the laboratory. Gamma ray spectroscopic studies of these cores were conducted with a large volume NaI crystal detector to observe γ rays of specific energies. Characteristic γ rays from various isotopes were identified confirming the presence and relative quantity of radon precursors in core samples. Geochemical analysis of cores was also conducted to measure presence and quantity of trace metals and radon precursors. Of 744 homes tested in Vanderburgh County from 2007 to 2013, 169 homes (22.7 percent) had elevated radon levels greater than 148 mBq L(-1) (4.0 pCi L(-1)). Additionally, 246 homes (33.1 percent) had measured radon levels of 74-145 mBq L(-1) (2.0-3.9 pCi L(-1)). About 80 percent of elevated radon levels greater than 148 mBq L(-1) (4.0 pCi L(-1)) are located in proximity to depositional contacts between the Dugger and Shelburn formations, or the

  9. Lineaments and fracture traces, Jennings County and Jefferson Proving Ground, Indiana

    Science.gov (United States)

    Greeman, T.K.

    1981-01-01

    Jennings and several adjacent counties are economically restricted "by inadequate water supplies. The North Vernon Water Utility, supplying more than 25 percent of Jennings County's population, obtains its water from the Vernon Fork Muscatatuck River, although streamflow is less than the average daily withdrawal 69 days of the year. The U.S. Army, Jefferson Proving Ground, pipes water more than 5 miles and lifts it 375 feet for fire protection. Another Jennings County utility pipes water more than 15 miles to rural domestic consumers unable to locate sufficient ground-water supplies.

  10. 2009-2012 Indiana Statewide Imagery and LiDAR Program: Maumee River Basin Counties

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — The counties comprised in this dataset have been chosen based on the relation to the Maumee River basin, a portion of the Lake Erie basin and correlated with the...

  11. 2009-2012 Indiana Statewide Imagery and LiDAR Program: Maumee River Basin Counties

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — The counties comprised in this dataset have been chosen based on the relation to the Maumee River basin, a portion of the Lake Erie basin and correlated with the...

  12. Hydrologic data collected in and around a surface coal mine, Clay and Vigo counties, Indiana, 1977-80

    Science.gov (United States)

    Bobo, Linda L.; Eikenberry, Stephen E.

    1982-01-01

    Few data are available for evaluating water-quality and other hydrologic properties in and around surface coal mines, particularly in areas where material having a high potential for acid-production is selectively buried. This report contains hydrologic data collected in an active coal mining area in Clay and Vigo Counties, Indiana, from September 1977 through February 1980. Methods of sampling and analysis used in collecting the data also are summarized. The data include field and laboratory measurements of water at 41 wells and 24 stream sites. Variables measured in the field include water temperature, specific conductance, pH, Eh, dissolved oxygen, ground-water levels, and streamflow; and in the laboratory, concentrations of major ions, alkalinity, hardness, trace elementsl, organic carbon, phosphorus, and dissolved solids. Other variables measured in the laboratory include ferrous iron concentration of water samples from selected wells, percent sulfur by weight and the potential acidity of core samples of reclaimed cast overburden, concentrations of elements absorbed on streambed materials, concentrations and particle size of suspended sediment in water, and populations and Shannon diversity indices of phytoplankton in water. Dissolved-solids concentrations and pH of ground water ranged from 173 to 5,130 milligrams per liter and from 6.1 to 8.9, respectively, and of surface water, from 120 to 4,100 milligrams per liter and from 6.1 to 8.8 respectively. 

  13. 78 FR 29657 - Prevailing Rate Systems; Definition of Vanderburgh County, Indiana, to a Nonappropriated Fund...

    Science.gov (United States)

    2013-05-21

    ... defining Vanderburgh County to the Christian- Montgomery wage area, we believe the organizational... Christian-Montgomery wage area. Based on our analysis of the organizational relationship and geographic... or after 30 days following publication of the final regulations. Regulatory Flexibility Act I certify...

  14. Huntington's Disease

    Science.gov (United States)

    Huntington's disease (HD) is an inherited disease that causes certain nerve cells in the brain to waste ... express emotions. If one of your parents has Huntington's disease, you have a 50 percent chance of ...

  15. The Development of Consumer-Driven Human Services Information Technology Initiatives: The Lake County Indiana Experience

    Directory of Open Access Journals (Sweden)

    Thomas W. Pavkov

    2003-01-01

    Full Text Available The Family Access Project will deploy innovative community empowerment, education, consensus building, and information system development strategies to strengthen community, ensure the efficient and effective delivery of needed services, and address the unique needs of families requiring public assistance from a host of public and private agencies in Lake County. The goal of the project is to enhance community life through improved care coordination by linking new technologies to the human service delivery process. Upon completion, the project will assist in the enhancement of community-based services through the development of rules of data transaction and data standards and the deploy-ment of a secure messaging/document exchange network. By putting technology in the hands of consumers we also hope to impact the economic development and workforce readiness goals set forth in our community's welfare to work programs. These innovations will require educational innovations in order to facilitate the use of technology by both provider and consumer end-users. Proposed innovations include tutorials related to data standards development, peer train-the-trainer training in the development and use of technology to support service system reforms; and ongoing support through a technical assistance clearinghouse and help desk.

  16. Modified level II streambed-scour analysis for structure I-70-60-5180 crossing Branch of McCracken Creek in Hendricks County, Indiana

    Science.gov (United States)

    Robinson, B.A.; Voelker, D.C.; Miller, R.L.

    1997-01-01

    Level II scour evaluations follow a process in which hydrologic, hydraulic, and sediment transport data are evaluated to calculate the depth of scour that may result when a given discharge is routed through a bridge opening. The results of the modified Level II analysis for structure 1-70-60-5180 on Interstate 70 crossing Branch of McCracken Creek in Hendricks County, Indiana, are presented. The site is near the town of Center Valley in the south-central part of Hendricks County. Scour depths were computed with the Water Surface PROfile model, version V050196, which incorporates the scour-calculation procedures outlined in Hydraulic Engineering Circular No. 18. Total scour depths at the piers were approximately 12.4 feet for the modeled discharge of 1,200 cubic feet per second and approximately 24.8 feet for the modeled discharge of 2,040 cubic feet per second.

  17. Modified level II streambed-scour analysis for structure I-69-64-4767 crossing Tippey Ditch in Grant County, Indiana

    Science.gov (United States)

    Robinson, B.A.; Voelker, D.C.; Miller, R.L.

    1997-01-01

    Level II scour evaluations follow a process in which hydrologic, hydraulic, and sediment transport data are evaluated to calculate the depth of scour that may result when a given discharge is routed through a bridge opening. The results of the modified Level II analysis for structure 1-69-64-4767 on Interstate 69 crossing Tippey Ditch in Grant County, Indiana, are presented. The site is near the town of Van Buren in the northeastern part of Grant County. Scour depths were computed with the Water Surface PROfile model, version V050196, which incorporates the scour-calculation procedures outlined in Hydraulic Engineering Circular No. 18. Total scour depths at the piers were approximately 11.9 feet for the modeled discharge of 2,000 cubic feet per second and approximately 19.8 feet for the modeled discharge of 3,400 cubic feet per second.

  18. Modified level II streambed-scour analysis for structure I-164-7-6974 crossing Schlensker Ditch in Vanderburgh County, Indiana

    Science.gov (United States)

    Miller, R.L.; Robinson, B.A.; Voelker, D.C.

    1997-01-01

    Level II scour evaluations follow a process in which hydrologic, hydraulic, and sedient-transport data are evaluated to calculate the depth of scour that may result when given discharge is routed through a bridge opening. the results of the modified Levell II analysis for structure I-674-7-6974 on Interstate 164 crossing Schlensker Ditch in Vanderburgh County, Indiana, are presented. The site is near the town of Daylight  in the northwestern part of Vanderburgh County. Scour depths were computed with the Water Surface PROfile model, version V050196, which incorporates the scour-calculation procedures outlined in Hydraulic Engineering Circular No. 18. Total scour depths at the piers were approximately 176 feet for the modeled discharge of 2,100  cubic feet per second and approximately 33.4 feet for the modeled discharge of 2,940 cubic feet per second.

  19. 78 FR 78720 - Approval and Promulgation of Air Quality Implementation Plans; Indiana; Disapproval of State...

    Science.gov (United States)

    2013-12-27

    ... LLC (ArcelorMittal) facility in Porter County, Indiana. This revision would remove the SO 2 emission... would amend 326 Indiana Administrative Code (IAC) 7-4-14, Porter County SO 2 Emission Limitations, by... identical flares. The limits for Lake County, Indiana, were established after the limits for Porter County...

  20. HYDRAULICS, Huntington COUNTY, IN

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — Recent developments in digital terrain and geospatial database management technology make it possible to protect this investment for existing and future projects to...

  1. A one-dimensional, steady-state, dissolved-oxygen model and waste-load assimilation study for Clear Creek, Monroe County, Indiana

    Science.gov (United States)

    Wilber, William G.; Crawford, Charles G.; Peters, J.G.; Girardi, F.P.

    1979-01-01

    A digital model calibrated to conditions in Clear Creek, Monroe County, IN, was used to develop alternatives for future waste loadings that would be compatible with Indiana stream water-quality standards defined for two critical hydrologic conditions, summer and winter low flows. The Winston Thomas wastewater-treatment facility is the only point-source waste load affecting the modeled reach of Clear Creek. A new waste-water-treatment facility under construction at Dillman Road (river mile 13.78) will replace the Winston Thomas wastewater-treatment facility (river mile 16.96) in 1980. Natural streamflow during the summer and annual 7-day, 10-year low flow is zero, so no benefit from dilution is provided. The model indicates that ammonia-nitrogen toxicity is the most significant factor affecting the stream water quality during summer and winter low flows. The ammonia-nitrogen concentration of the wastewater effluent exceeds the maximum total ammonia-nitrogen concentration of 2.5 milligrams per liter for summer months (June through August) and 4.0 milligrams per liter for winter months (November through March) required for Indiana streams. Nitrification, benthic-oxygen demand, and algal respiration were the most significant factors affecting the dissolved-oxygen concentration in Clear Creek during the model calibration. Nitrification should not significantly affect the dissolved-oxygen concentration in Clear Creek during summer low flows when the ammonia-nitrogen toxicity standards are met. (USGS)

  2. An Examination of Teachers Teaching Teachers Staff Development Model in Southwest Dubois County. Prepared for the Southwest Dubois County School Corporation, Huntingburg, Indiana.

    Science.gov (United States)

    Gilman, David A.; Miller, Melinda

    This study investigated how the Teachers Teaching Teachers project influenced the attitudes and beliefs of public school teachers and administrators. Eighteen educators from the Southwest Dubois School Corporation, Huntingburg, Indiana, participated in the project, which lasted from November 1987 to May 1988. The results support the effectiveness…

  3. Coal resources of Indiana

    Science.gov (United States)

    Spencer, Frank Darwyn

    1953-01-01

    The Indiana coal field forms the eastern edge of the eastern interior coal basin, which is near some of the most densely populated and highly productive manufacturing areas of the United States. (See fig. 1. ) For this reason Indiana coal reserves are an important State and National asset. In dollar value the coal mining industry is the largest of Indiana's natural-resource-producing industries. The total value of coil production for the year 1950 was more than 100 million dollars, or more than that of all other natural-resource industries in the State combined. As estimated herein, the original coal reserves of Indiana total 37,293 million tons, of which 27,320 million tons is contained in beds more than 42 inches thick; 7,632 million tons in beds 28 to 49. inches thick; and 2,341 million tons in beds 14 to 28 inches thick. The remaining reserves as of January 1951, total 35,806 million tons, of which 18,779 million tons is believed to be recoverable. The distribution of the reserves in these several categories is summarized by counties in table 1. Of the total original reserves of 37,293 million tons, 6,355 million tons can be classified as measured; 8,657 million tons as indicated; and 22,281 million tons as inferred. Strippable reserves constitute 3,524 million tons, or 9.5 percent of the total original reserves. The distribution of the strippable and nonstrippable original reserves is summarized in tables 2 and 3 by counties and by several categories, according to the thickness of the beds and the relative abundance and reliability of the information available for preparing the estimates. The distribution of the estimated 18,779 million tons of recoverable strippable and nonstrippable reserves in Indiana is further summarized by counties in table 4, and the information is presented graphically in figures 2 and 3. The tables i to 4 and figures 2 and 3 include beds in the 14- to 28-inch category, because thin beds have been mined in many places. However, many

  4. Huntington's disease

    DEFF Research Database (Denmark)

    Hjermind, Lena Elisabeth; Law, Ian; Jønch, Aia

    2011-01-01

    In this open-label pilot study, the authors evaluated the effect of memantine on the distribution of brain glucose metabolism in four Huntington's disease (HD) patients as determined by serial 18-fluoro-deoxyglucose [F(18)]FDG-PET scans over a period of 3-4 months (90-129 days, with one patient...

  5. Huntington disease

    Science.gov (United States)

    ... President of the Florida Society of Neurology (FSN). Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team. Huntington's Disease Read more Latest Health News Read more Health ...

  6. Learning about Huntington's Disease

    Science.gov (United States)

    ... Mouse Models Of Huntington's Disease 1998 News Release Learning About Huntington's Disease What do we know about ... and treatment information. Hosted by the Dolan DNA Learning Center at Cold Spring Harbor Laboratory. Huntington's Outreach ...

  7. 75 FR 8428 - The Indiana Rail Road Company-Abandonment Exemption-in Martin and Lawrence Counties, IN; CSX...

    Science.gov (United States)

    2010-02-24

    ... Mayor; Douglas England, City of New Albany Mayor; David Branneman, Lawrence County Tourism Commission... posted on the Board's Web site. This action will not significantly affect either the quality of the human environment or the conservation of energy resources. Dated: February 18, 2010. By the Board, Rachel...

  8. Occurrence and attempted mitigation of carbon dioxide in a home constructed on reclaimed coal-mine spoil, Pike County, Indiana

    Science.gov (United States)

    Robinson, Bret A.

    2010-01-01

    In recent years carbon dioxide intrusion has become recognized as a potentially serious health threat where homes are constructed on or near reclaimed surface coal mines. When carbon dioxide invades the living space of a home, it can collect near the floor, displace the oxygen there, and produce an oxygen-deficient environment. In this investigation, several lines of inquiry were pursued to determine the environmental factors that most influence carbon dioxide intrusion at a Pike County, Ind., home where this phenomenon is known to occur. It was found that carbon dioxide intrusion events at the home are most closely tied to rapid drops in barometric pressure and rainfall. Other researchers have shown that windy conditions and periods of cold weather also can contribute to soil-gas intrusion to structures. From this, a conceptual model was developed to illustrate the influence of these four meteorological conditions. Additionally, three mitigation methods-block-wall depressurization, block-wall and sub-slab depressurization, and block-wall and sub-slab pressurization-were applied successively to the study-site home, and environmental data were collected to evaluate the effectiveness of each mitigation method. In each case, it was found that these methods did not ensure a safe environment when meteorological conditions were favorable for carbon dioxide intrusion.

  9. Assessment of Surface Water Contamination from Coalbed Methane Fracturing-Derived Volatile Contaminants in Sullivan County, Indiana, USA.

    Science.gov (United States)

    Meszaros, Nicholas; Subedi, Bikram; Stamets, Tristan; Shifa, Naima

    2017-07-14

    There is a growing concern over the contamination of surface water and the associated environmental and public health consequences from the recent proliferation of hydraulic fracturing in the USA. Petroleum hydrocarbon-derived contaminants of concern [benzene, toluene, ethylbenzene, and xylenes (BTEX)] and various dissolved cations and anions were spatially determined in surface waters around 15 coalbed methane fracking wells in Sullivan County, IN, USA. At least one BTEX compound was detected in 69% of sampling sites (n = 13) and 23% of sampling sites were found to be contaminated with all of the BTEX compounds. Toluene was the most common BTEX compound detected across all sampling sites, both upstream and downstream from coalbed methane fracking wells. The average concentration of toluene at a reservoir and its outlet nearby the fracking wells was ~2× higher than other downstream sites. However, one of the upstream sites was found to be contaminated with BTEX at similar concentrations as in a reservoir site nearby the fracking well. Calcium (~60 ppm) and sulfates (~175 ppm) were the dominant cations and anions, respectively, in surface water around the fracking sites. This study represents the first report of BTEX contamination in surface water from coalbed methane hydraulic fracturing wells.

  10. 76 FR 59600 - Approval and Promulgation of Air Quality Implementation Plans; Indiana; Redesignation of Lake and...

    Science.gov (United States)

    2011-09-27

    ...; Redesignation of Lake and Porter Counties to Attainment of the Fine Particulate Matter Standard AGENCY... Management (IDEM) requested redesignation of the Lake and Porter Counties, Indiana portion (Lake and Porter... Porter Counties, along with related Indiana State Implementation Plan (SIP) revisions, including the...

  11. 76 FR 76302 - Approval and Promulgation of Air Quality Implementation Plans; Indiana; Redesignation of Lake and...

    Science.gov (United States)

    2011-12-07

    ...; Redesignation of Lake and Porter Counties to Attainment of the Fine Particulate Matter Standard AGENCY... affecting Lake and Porter Counties and the State of Indiana for the 1997 annual fine particulate matter (PM... State of Indiana for the redesignation of Lake and Porter Counties to attainment of the 1997 annual PM...

  12. Coal Fields - COAL_SPRINGFIELD_THICKNESS_IN: Thickness Ranges of the Springfield Coal Member (Petersburg Formation, Pennsylvanian) in West-Central Indiana (Indiana Geological Survey, 1:126,720, Polygon Coverage)

    Data.gov (United States)

    NSGIC State | GIS Inventory — From 1985 to 1994, a series of reports on coal resources of selected counties in Indiana was published as part of the Special Report series of the Indiana Geological...

  13. Coal Fields - COAL_SPRINGFIELD_ELEVATION_IN: Elevation Ranges of the Springfield Coal Member (Petersburg Formation, Pennsylvanian) in West-Central Indiana (Indiana Geological Survey, 1:126,720, Polygon Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — A series of reports on coal resources of selected counties in Indiana were published as part of the Special Report series of the Indiana Geological Survey between...

  14. Coal Fields - COAL_DANVILLE_THICKNESS_IN: Thickness Ranges of the Danville Coal Member (Dugger Formation, Pennsylvanian) in West-Central Indiana (Indiana Geological Survey, 1:126,720, Polygon Shapefile)

    Data.gov (United States)

    NSGIC State | GIS Inventory — From 1985 to 1994, a series of reports on coal resources of selected counties in Indiana was published as part of the Special Report series of the Indiana Geological...

  15. Coal Fields - COAL_HOUCHIN_CREEK_ELEVATION_IN: Elevation Ranges of the Houchin Creek Coal Member (Petersburg Formation, Pennsylvanian) in West-Central Indiana (Indiana Geological Survey, 1:126,720, Polygon Coverage)

    Data.gov (United States)

    NSGIC State | GIS Inventory — From 1985 to 1994, a series of reports on coal resources of selected counties in Indiana was published as part of the Special Report series of the Indiana Geological...

  16. Coal Fields - COAL_HYMERA_THICKNESS_IN: Thickness Ranges of the Hymera Coal Member (Dugger Formation, Pennsylvanian) in West-Central Indiana (Indiana Geological Survey, 1:126,720, Polygon Shapefile)

    Data.gov (United States)

    NSGIC State | GIS Inventory — From 1988 to 1994, a series of reports on coal resources of selected counties in Indiana was published as part of the Special Report series of the Indiana Geological...

  17. Coal Fields - COAL_COLCHESTER_ELEVATION_IN: Elevation Ranges of the Colchester Coal Member (Linton Formation, Pennsylvanian) in West-Central Indiana (Indiana Geological Survey, 1:126,720, Polygon Shapefile)

    Data.gov (United States)

    NSGIC State | GIS Inventory — From 1985 to 1994, a series of reports on coal resources of selected counties in Indiana was published as part of the Special Report series of the Indiana Geological...

  18. Coal Fields - COAL_DANVILLE_ELEVATION_IN: Elevation Ranges of the Danville Coal Member (Dugger Formation, Pennsylvanian) in West-Central Indiana (Indiana Geological Survey, 1:126,720, Polygon Shapefile)

    Data.gov (United States)

    NSGIC State | GIS Inventory — From 1985 to 1994, a series of reports on coal resources of selected counties in Indiana was published as part of the Special Report series of the Indiana Geological...

  19. Coal Fields - COAL_HYMERA_ELEVATION_IN: Elevation Ranges of the Hymera Coal Member (Dugger Formation, Pennsylvanian) in West-Central Indiana (Indiana Geological Survey, 1:126,720, Polygon Shapefile)

    Data.gov (United States)

    NSGIC State | GIS Inventory — From 1985 to 1994, a series of reports on coal resources of selected counties in Indiana was published as part of the Special Report series of the Indiana Geological...

  20. Coal Fields - COAL_COLCHESTER_ELEVATION_IN: Elevation Ranges of the Colchester Coal Member (Linton Formation, Pennsylvanian) in West-Central Indiana (Indiana Geological Survey, 1:126,720, Polygon Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — From 1985 to 1994, a series of reports on coal resources of selected counties in Indiana was published as part of the Special Report series of the Indiana Geological...

  1. 76 FR 47287 - Indiana Disaster Number IN-00037

    Science.gov (United States)

    2011-08-04

    ... ADMINISTRATION Indiana Disaster Number IN-00037 AGENCY: U.S. Small Business Administration. ACTION: Amendment 2... major disaster declaration for Private Non-Profit organizations in the State of Indiana, dated 06/23... Counties: Vermillion, Wayne. All other information in the original declaration remains unchanged. (Catalog...

  2. Psychopathology in Huntington's disease

    NARCIS (Netherlands)

    Duijn, Erik van

    2010-01-01

    Dit proefschrift begint met een overzichtsartikel van oorspronkelijke onderzoek naar psychopathologie bij mutatiedragers voor de ziekte van Huntington. Aansluitend worden de resultaten van een cohortstudie naar de aanwezigheid en ernst van psychopathologie bij mensen met de ziekte van Huntington in

  3. Psychopathology in Huntington's disease

    NARCIS (Netherlands)

    Duijn, Erik van

    2010-01-01

    Dit proefschrift begint met een overzichtsartikel van oorspronkelijke onderzoek naar psychopathologie bij mutatiedragers voor de ziekte van Huntington. Aansluitend worden de resultaten van een cohortstudie naar de aanwezigheid en ernst van psychopathologie bij mensen met de ziekte van Huntington in

  4. FLOODPLAIN MAPPING, Huntington COUNTY, IN

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — The Floodplain Mapping study deliverables depict and quantify the flood risks for the study area. The primary risk classifications used are the...

  5. Indiana Health Information Exchange

    Science.gov (United States)

    The Indiana Health Information Exchange is comprised of various Indiana health care institutions, established to help improve patient safety and is recognized as a best practice for health information exchange.

  6. 75 FR 12087 - Determination of Attainment, Approval and Promulgation of Air Quality Implementation Plans; Indiana

    Science.gov (United States)

    2010-03-12

    ... several related actions under the Clean Air Act (CAA) affecting the Indiana portion (Lake and Porter... Indiana to exempt sources of Nitrogen Oxides (NO X ) in Lake and Porter Counties from CAA Reasonably... request for Lake and Porter Counties, also published in today's Federal Register, the Chicago-Gary-Lake...

  7. Huntington's disease phenocopy syndromes.

    Science.gov (United States)

    Wild, Edward J; Tabrizi, Sarah J

    2007-12-01

    Patients presenting with features of Huntington's disease but lacking the causative genetic expansion can be challenging diagnostically. The differential diagnosis of such Huntington's disease phenocopy syndromes has not recently been reviewed. Cohort studies have established the relative frequencies of known Huntington's disease phenocopy syndromes, whereas newly described ones have been characterized genetically, clinically, radiologically and pathologically. About 1% of suspected Huntington's disease cases emerge as phenocopy syndromes. Such syndromes are clinically important in their own right but may also shed light on the pathogenesis of Huntington's disease. Huntington's disease produces a range of clinical phenotypes, and the range of syndromes that may be responsible for Huntington's disease phenocopies is correspondingly wide. Cohort studies have established that, while the majority of phenocopy patients remain undiagnosed, in those patients where a genetic diagnosis is reached the commonest causes are SCA17, Huntington's disease-like syndrome 2 (HDL2), familial prion disease and Friedreich's ataxia. We review the features of the reported genetic causes of Huntington's disease phenocopy syndromes, including HDL1-3, SCA17, familial prion disease, spinocerebellar ataxias, dentatorubral-pallidoluysian atrophy, chorea-acanthocytosis and iron-accumulation disorders. We present an evidence-based framework for the genetic testing of Huntington's disease phenocopy cases.

  8. Mapping energy poverty in Huntington, West Virginia

    Science.gov (United States)

    Callicoat, Elizabeth Anne

    Energy poverty is a growing phenomenon culminating from the combination of low to mid household income, deteriorating housing structures and rising household energy costs. Energy prices are increasing for all households, but the burden is proportionally larger for those with low to mid income. These groups must sacrifice to afford energy, and are often unable or do not have the autonomy to make structural improvements, especially if they rent their home. Data on residential dwellings from the Cabell County Tax Assessor's Office was used within a geographic information system to map where energy poverty likely exists within the city limits of Huntington, WV. It was found that one fifth of Huntington households are at a high risk of energy poverty, primarily located across the northern section of the city and in the center, surrounding Marshall University, Downtown and Cabell Huntington Hospital.

  9. Koltunud Indiana Jones / Kutt Kommel

    Index Scriptorium Estoniae

    Kommel, Kutt

    2008-01-01

    Steven Spielbergi neljas Indiana Jones'i film Harrison Fordiga nimiosas "Indiana Jones ja kristallpealuu kuningriik" ("Indiana Jones and the Kingdom of the Crystal Skull") : Ameerika Ühendriigid 2008

  10. Indiana Dzhons vozvrashtshajetsja / Melor Sturua

    Index Scriptorium Estoniae

    Sturua, Melor

    2008-01-01

    22 mail esilinastub Steven Spielbergi järjekordne Indiana Jones'i film, kaasstsenaristiks ja produtsendiks George Lucas ja Harrison Fordiga nimiosas "Indiana Jones ja kristallpealuu kuningriik" ("Indiana Jones and the Kingdom of the Crystal Skull")

  11. Indiana Dzhons vozvrashtshajetsja / Melor Sturua

    Index Scriptorium Estoniae

    Sturua, Melor

    2008-01-01

    22 mail esilinastub Steven Spielbergi järjekordne Indiana Jones'i film, kaasstsenaristiks ja produtsendiks George Lucas ja Harrison Fordiga nimiosas "Indiana Jones ja kristallpealuu kuningriik" ("Indiana Jones and the Kingdom of the Crystal Skull")

  12. Koltunud Indiana Jones / Kutt Kommel

    Index Scriptorium Estoniae

    Kommel, Kutt

    2008-01-01

    Steven Spielbergi neljas Indiana Jones'i film Harrison Fordiga nimiosas "Indiana Jones ja kristallpealuu kuningriik" ("Indiana Jones and the Kingdom of the Crystal Skull") : Ameerika Ühendriigid 2008

  13. Mercury in Precipitation in Indiana, January 2004-December 2005

    Science.gov (United States)

    Risch, Martin R.; Fowler, Kathleen K.

    2008-01-01

    Mercury in precipitation was monitored during 2004-2005 at five locations in Indiana as part of the National Atmospheric Deposition Program-Mercury Deposition Network (NADP-MDN). Monitoring stations were operated at Roush Lake near Huntington, Clifty Falls State Park near Madison, Fort Harrison State Park near Indianapolis, Monroe County Regional Airport near Bloomington, and Indiana Dunes National Lakeshore near Porter. At these monitoring stations, precipitation amounts were measured continuously and weekly samples were collected for analysis of mercury by methods achieving detection limits as low as 0.05 ng/L (nanograms per liter). Wet deposition was computed as the product of mercury concentration and precipitation. The data were analyzed for seasonal patterns, temporal trends, and geographic differences. In the 2 years, 520 weekly samples were collected at the 5 monitoring stations and 448 of these samples had sufficient precipitation to compute mercury wet deposition. The 2-year mean mercury concentration at the five monitoring stations (normalized to the sample volume) was 10.6 ng/L. As a reference for comparison, the total mercury concentration in 41 percent of the samples analyzed was greater than the statewide Indiana water-quality standard for mercury (12 ng/L, protecting aquatic life) and 99 percent of the concentrations exceeded the most conservative Indiana water-quality criterion (1.3 ng/L, protecting wild mammals and birds). The normalized annual mercury concentration at Clifty Falls in 2004 was the fourth highest in the NADP-MDN in eastern North America that year. In 2005, the mercury concentrations at Clifty Falls and Indiana Dunes were the ninth highest in the NADP-MDN in eastern North America. At the five monitoring stations during the study period, the mean weekly total mercury deposition was 0.208 ug/m2 (micrograms per square meter) and mean annual total mercury deposition was 10.8 ug/m2. The annual mercury deposition at Clifty Falls in 2004

  14. [The Henry E. Huntington Library.

    Science.gov (United States)

    Abraham, Terry

    The biographical sketch of Henry E. Huntington includes a description of the establishment of the Huntington Library and the purpose and scope of its collection. Although this is a free and public library, its use is restricted to qualified scholars having legitimate research needs. Photographic techniques were developed at the Huntington Library…

  15. A one-dimensional, steady-state, dissolved-oxygen model and waste-load assimilation study for West Fork Blue River, Washington County, Indiana

    Science.gov (United States)

    Peters, James G.; Wilber, W.G.; Crawford, Charles G.; Girardi, F.P.

    1979-01-01

    A digital computer model calibrated to observe stream conditions was used to evaluate water quality in West Fork Blue River, Washington County, IN. Instream dissolved-oxygen concentration averaged 96.5% of saturation at selected sites on West Fork Blue River during two 24-hour summer surveys. This high dissolved-oxygen concentration reflects small carbonaceous and nitrogenous waste loads; adequate dilution of waste by the stream; and natural reaeration. Nonpoint source waste loads accounted for an average of 53.2% of the total carbonaceous biochemical-oxygen demand and 90.2% of the nitrogenous biochemical-oxygen demand. Waste-load assimilation was studiedfor critical summer and winter low flows. Natural streamflow for these conditions was zero, so no benefit from dilution was provided. The projected stream reaeration capacity was not sufficient to maintain the minimum daily dissolved-oxygen concentration (5 milligrams per liter) in the stream with current waste-discharge restrictions. During winter low flow, ammonia toxicity, rather than dissolved-oxygen concentration, was the limiting water-quality criterion downstream from the Salem wastewater-treatment facility. (USGS)

  16. Implications of Changing Ethnic-Group Representation in Indiana's Population. Part 1: Highlights and Summary. Manpower Report 86-2.

    Science.gov (United States)

    Lisack, J. P.; Shell, Kevin D.

    From 1970 to 1980, Indiana's population grew 5.7 percent, with the white population growing less than 4 percent as opposed to a 30 percent growth rate for minority groups. Nearly 64.4 of the state's minority population resided in Marion and Lake counties as of 1980. Except for Asian Americans, Indiana residents who belong to ethnic minority groups…

  17. Roost tree selection and use by the Indiana Bat on Walnut Creek National Wildlife Refuge: First year progress report

    Data.gov (United States)

    US Fish and Wildlife Service, Department of the Interior — The Indiana bat (Myotis sodalis) was first reported in Iowa in 1959 (Bowles, 1975). This first record was of three hibernating individuals, in a Dubuque County cave....

  18. Indiana Jones ja kristallpealuu kuningriik / Jaanus Noormets

    Index Scriptorium Estoniae

    Noormets, Jaanus

    2008-01-01

    Steven Spielbergi neljas Indiana Jones'i film Harrison Fordiga nimiosas "Indiana Jones ja kristallpealuu kuningriik" ("Indiana Jones and the Kingdom of the Crystal Skull") : Ameerika Ühendriigid 2008

  19. Indiana protiv KGB / Anna Fedina, Pjotr Obraztsov

    Index Scriptorium Estoniae

    Fedina, Anna

    2008-01-01

    Steven Spielbergi neljas Indiana Jones'i film Harrison Fordiga nimiosas "Indiana Jones ja kristallpealuu kuningriik" ("Indiana Jones and the Kingdom of the Crystal Skull") : Ameerika Ühendriigid 2008

  20. Indiana Jones ja kristallpealuu kuningriik / Jaanus Noormets

    Index Scriptorium Estoniae

    Noormets, Jaanus

    2008-01-01

    Steven Spielbergi neljas Indiana Jones'i film Harrison Fordiga nimiosas "Indiana Jones ja kristallpealuu kuningriik" ("Indiana Jones and the Kingdom of the Crystal Skull") : Ameerika Ühendriigid 2008

  1. Indiana protiv KGB / Anna Fedina, Pjotr Obraztsov

    Index Scriptorium Estoniae

    Fedina, Anna

    2008-01-01

    Steven Spielbergi neljas Indiana Jones'i film Harrison Fordiga nimiosas "Indiana Jones ja kristallpealuu kuningriik" ("Indiana Jones and the Kingdom of the Crystal Skull") : Ameerika Ühendriigid 2008

  2. Environmental Assessment of the Muscatatuck Urban Training Center near Butlerville, Indiana, October and November 2005

    Science.gov (United States)

    Risch, Martin R.; Ulberg, Amanda L.; Robinson, Bret A.

    2007-01-01

    An environmental assessment of the Muscatatuck Urban Training Center near Butlerville in Jennings County, Indiana, was completed during October and November 2005. As part of the Department of Defense Earth Science Program, the U.S. Geological Survey collected information about environmental conditions at the 825-acre former State of Indiana mental health facility prior to its conversion by the Indiana National Guard into an urban training center. The assessment was designed to investigate the type and extent of potential contamination associated with historical activities in selected areas of the facility.

  3. Clinical neurogenetics: huntington disease.

    Science.gov (United States)

    Bordelon, Yvette M

    2013-11-01

    Huntington disease (HD) is an autosomal dominant, adult-onset, progressive neurodegenerative disease characterized by the triad of abnormal movements (typically chorea), cognitive impairment, and psychiatric problems. It is caused by an expanded CAG repeat in the gene encoding the protein huntingtin on chromosome 4 and causes progressive atrophy of the striatum as well as cortical and other extrastriatal structures. Genetic testing has been available since 1993 to confirm diagnosis in affected adults and for presymptomatic testing in at-risk individuals. This review covers HD signs, symptoms, and pathophysiology; current genetic testing issues; and current and future treatment strategies.

  4. Distribution of odonates (dragonflies and damselflies) in the Indiana Dunes National Lakeshore and nearby lands

    Science.gov (United States)

    Smolka, George E.; Stewart, Paul M.; Swinford, Thomas O.

    1999-01-01

    From 1993 to 1997, 60 species of Anisoptera (dragonflies) and Zygoptera (damselflies) were found in Lake and Porter Counties, Indiana, including Indiana Dunes National Lakeshore, in contrast to 34 species that were recorded historically from this region. We added 17 new species to Lake County's odonate records and 39 new species to the 5 previously recorded in Porter County. Several regionally rare species were collected: Aeshna clepsydra, Enallagma cyathigerum, and Leucorrhina frigida. Nine species listed in the historical records were missing from our collections: Hetaerina americana, Calopteryx aequabilis, Nehalennia irene, Arigomphus furcifer, Argia fumipennis violacea, Gomphus spicatus, Epitheca princeps, Libellula exusta, and Sympetrum semicinctum. These nine species have either declined in the area or they may be found in other habitats after further study. Because few odonate surveys were conducted in northwest Indiana in the past, a poor baseline exists for comparisons of temporal trends in odonate diversity.

  5. Indiana in the World, the World in Indiana: Exploring Indiana's International Connections.

    Science.gov (United States)

    Koben, Rise, Ed.

    In an era of globalization, Indiana's cultural, geographic, and economic relationships are becoming more prevalent. This guide is designed for use in middle and high school classrooms to encourage teachers and students to examine their own community's international relationships. The materials were developed by Indiana teachers and are organized…

  6. Huntington's disease presenting as amyotrophic lateral sclerosis.

    LENUS (Irish Health Repository)

    Phukan, Julie

    2010-08-01

    We present the clinical, electrophysiological and molecular genetic findings of a 58-year-old male with genetically confirmed Huntington\\'s disease (HD) and concurrent clinically definite ALS by El Escorial criteria. The patient presented with asymmetric upper limb amyotrophy and weakness, and subsequently developed chorea and cognitive change. Genetic testing confirmed the presence of expanded trinucleotide repeats in huntingtin, consistent with a diagnosis of Huntington\\'s disease. This case confirms the rare coexistence of Huntington\\'s disease and motor neuron degeneration.

  7. What is HD - Huntington's Disease?

    Science.gov (United States)

    ... the person less able to work at their customary level and less functional in their regular activities ... not is intensely personal and there is no "right" answer. The Huntington's Disease Society of America recommends ...

  8. Stages of Huntington's Disease (HD)

    Science.gov (United States)

    ... the person less able to work at their customary level and less functional in their regular activities ... not is intensely personal and there is no "right" answer. The Huntington's Disease Society of America recommends ...

  9. Natural history of Huntington disease.

    Science.gov (United States)

    Dorsey, E Ray; Beck, Christopher A; Darwin, Kristin; Nichols, Paige; Brocht, Alicia F D; Biglan, Kevin M; Shoulson, Ira

    2013-12-01

    Understanding the natural history of Huntington disease will inform patients and clinicians on the disease course and researchers on the design of clinical trials. To determine the longitudinal change in clinical features among individuals with Huntington disease compared with controls. Prospective, longitudinal cohort study at 44 research sites in Australia (n = 2), Canada (n =4), and the United States (n = 38). Three hundred thirty-four individuals with clinically manifest Huntington disease who had at least 3 years of annually accrued longitudinal data and 142 controls consisting of caregivers and spouses who had no genetic risk of Huntington disease. Change in movement, cognition, behavior, and function as measured by the Unified Huntington's Disease Rating Scale, the Mini-Mental State Examination, and vital signs. Total motor score worsened by 3.0 points (95% CI, 2.5-3.4) per year and chorea worsened by 0.3 point per year (95% CI, 0.1-0.5). Cognition declined by 0.7 point (95% CI, 0.6-0.8) per year on the Mini-Mental State Examination. Behavior, as measured by the product of frequency and severity score on the Unified Huntington's Disease Rating Scale, worsened by 0.6 point per year (95% CI, 0.0-1.2). Total functional capacity declined by 0.6 point per year (95% CI, 0.5-0.7). Compared with controls, baseline body mass index was lower in those with Huntington disease (25.8 vs 28.8; P Huntington disease all declined in a monotonic manner. These data quantify the natural history of the disease and may inform the design of trials aimed at reducing its burden. clinicaltrials.gov Identifier: NCT00313495.

  10. Huntington's disease in children.

    Science.gov (United States)

    Letort, Derek; Gonzalez-Alegre, Pedro

    2013-01-01

    Huntington's disease (HD) is a dominantly inherited, fatal neurodegenerative disease. This incurable illness is characterized by a triad of a movement disorder, cognitive decline and psychiatric manifestations. Although most patients with HD have disease onset in the adult years, a small but significant proportion present with pediatric HD. It has been long known that patients with early-onset HD commonly exhibit prominent parkinsonism, known as the Westphal variant of HD. However, even among patients with pediatric HD there are differential clinical features depending on the age of onset, with younger patients frequently presenting diagnostic challenges. In his chapter, the characteristics of patients with childhood- and adolescence-onset HD are discussed, focusing on the differential clinical features that can aid the clinical reach a correct diagnosis, the indications and rational use of genetic testing and the currently available options for symptomatic treatment.

  11. Traffic Analysis Zones - TRAFFIC_ZONES_TIGER00_IN: Indiana Traffic Analysis Zones (U.S. Census Bureau, 1:100,000, Polygon Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — TRAFFIC_ZONES_TIGER00_IN contains traffic analysis zones in Indiana identified by the US Bureau of the Census. Vigo, Madison, and Monroe counties were not available...

  12. Clean coal initiatives in Indiana

    Science.gov (United States)

    Bowen, B.H.; Irwin, M.W.; Sparrow, F.T.; Mastalerz, Maria; Yu, Z.; Kramer, R.A.

    2007-01-01

    Purpose - Indiana is listed among the top ten coal states in the USA and annually mines about 35 million short tons (million tons) of coal from the vast reserves of the US Midwest Illinois Coal Basin. The implementation and commercialization of clean coal technologies is important to the economy of the state and has a significant role in the state's energy plan for increasing the use of the state's natural resources. Coal is a substantial Indiana energy resource and also has stable and relatively low costs, compared with the increasing costs of other major fuels. This indigenous energy source enables the promotion of energy independence. The purpose of this paper is to outline the significance of clean coal projects for achieving this objective. Design/methodology/approach - The paper outlines the clean coal initiatives being taken in Indiana and the research carried out at the Indiana Center for Coal Technology Research. Findings - Clean coal power generation and coal for transportation fuels (coal-to-liquids - CTL) are two major topics being investigated in Indiana. Coking coal, data compilation of the bituminous coal qualities within the Indiana coal beds, reducing dependence on coal imports, and provision of an emissions free environment are important topics to state legislators. Originality/value - Lessons learnt from these projects will be of value to other states and countries.

  13. Treatment of Huntington's disease.

    Science.gov (United States)

    Frank, Samuel

    2014-01-01

    Huntington's disease (HD) is a dominantly inherited progressive neurological disease characterized by chorea, an involuntary brief movement that tends to flow between body regions. HD is typically diagnosed based on clinical findings in the setting of a family history and may be confirmed with genetic testing. Predictive testing is available to family members at risk, but only experienced clinicians should perform the counseling and testing. Multiple areas of the brain degenerate, mainly involving the neurotransmitters dopamine, glutamate, and γ-aminobutyric acid. Although pharmacotherapies theoretically target these neurotransmitters, few well-conducted trials for symptomatic interventions have yielded positive results and current treatments have focused on the motor aspects of HD. Tetrabenazine is a dopamine-depleting agent that may be one of the more effective agents for reducing chorea, although it has a risk of potentially serious adverse effects. Some newer neuroleptic agents, such as olanzapine and aripiprazole, may have adequate efficacy with a more favorable adverse effect profile than older neuroleptic agents for treating chorea and psychosis. There are no current treatments to change the course of HD, but education and symptomatic therapies can be effective tools for clinicians to use with patients and families affected by HD.

  14. Dopamine and Huntington's disease.

    Science.gov (United States)

    Schwab, Laetitia C; Garas, Shady N; Garas, Shaady N; Drouin-Ouellet, Janelle; Mason, Sarah L; Stott, Simon R; Barker, Roger A

    2015-04-01

    Huntington's disease (HD) is an incurable, inherited, progressive neurodegenerative disorder that is defined by a combination of motor, cognitive and psychiatric features. Pre-clinical and clinical studies have demonstrated an important role for the dopamine (DA) system in HD with dopaminergic dysfunction at the level of both DA release and DA receptors. It is, therefore, not surprising that the drug treatments most commonly used in HD are anti-dopaminergic agents. Their use is based primarily on the belief that the characteristic motor impairments are a result of overactivation of the central dopaminergic pathways. While this is a useful starting place, it is clear that the behavior of the central dopaminergic pathways is not fully understood in this condition and may change as a function of disease stage. In addition, how abnormalities in dopaminergic systems may underlie some of the non-motor features of HD has also been poorly investigated and this is especially important given the greater burden these place on the patients' and families' quality of life. In this review, we discuss what is known about central dopaminergic pathways in HD and how this informs us about the mechanisms of action of the dopaminergic therapies used to treat it. By doing so, we will highlight some of the paradoxes that exist and how solving them may reveal new insights for improved treatment of this currently incurable condition, including the possibility that such drugs may even have effects on disease progression and pathogenesis.

  15. Huntington's Disease and Mitochondria.

    Science.gov (United States)

    Jodeiri Farshbaf, Mohammad; Ghaedi, Kamran

    2017-06-21

    Huntington's disease (HD) as an inherited neurodegenerative disorder leads to neuronal loss in striatum. Progressive motor dysfunction, cognitive decline, and psychiatric disturbance are the main clinical symptoms of the HD. This disease is caused by expansion of the CAG repeats in exon 1 of the huntingtin which encodes Huntingtin protein (Htt). Various cellular and molecular events play role in the pathology of HD. Mitochondria as important organelles play crucial roles in the most of neurodegenerative disorders like HD. Critical roles of the mitochondria in neurons are ATP generation, Ca(2+) buffering, ROS generation, and antioxidant activity. Neurons as high-demand energy cells closely related to function, maintenance, and dynamic of mitochondria. In the most neurological disorders, mitochondrial activities and dynamic are disrupted which associate with high ROS level, low ATP generation, and apoptosis. Accumulation of mutant huntingtin (mHtt) during this disease may evoke mitochondrial dysfunction. Here, we review recent findings to support this hypothesis that mHtt could cause mitochondrial defects. In addition, by focusing normal huntingtin functions in neurons, we purpose mitochondria and Huntingtin association in normal condition. Moreover, mHtt affects various cellular signaling which ends up to mitochondrial biogenesis. So, it could be a potential candidate to decline ATP level in HD. We conclude how mitochondrial biogenesis plays a central role in the neuronal survival and activity and how mHtt affects mitochondrial trafficking, maintenance, integrity, function, dynamics, and hemostasis and makes neurons vulnerable to degeneration in HD.

  16. Neuroimaging in Huntington's disease.

    Science.gov (United States)

    Niccolini, Flavia; Politis, Marios

    2014-06-28

    Huntington's disease (HD) is a progressive and fatal neurodegenerative disorder caused by an expanded trinucleotide CAG sequence in huntingtin gene (HTT) on chromosome 4. HD manifests with chorea, cognitive and psychiatric symptoms. Although advances in genetics allow identification of individuals carrying the HD gene, much is still unknown about the mechanisms underlying the development of overt clinical symptoms and the transitional period between premanifestation and manifestation of the disease. HD has no cure and patients rely only in symptomatic treatment. There is an urgent need to identify biomarkers that are able to monitor disease progression and assess the development and efficacy of novel disease modifying drugs. Over the past years, neuroimaging techniques such as magnetic resonance imaging (MRI) and positron emission tomography (PET) have provided important advances in our understanding of HD. MRI provides information about structural and functional organization of the brain, while PET can detect molecular changes in the brain. MRI and PET are able to detect changes in the brains of HD gene carriers years ahead of the manifestation of the disease and have also proved to be powerful in assessing disease progression. However, no single technique has been validated as an optimal biomarker. An integrative multimodal imaging approach, which combines different MRI and PET techniques, could be recommended for monitoring potential neuroprotective and preventive therapies in HD. In this article we review the current neuroimaging literature in HD.

  17. Libraries in Indiana: MedlinePlus

    Science.gov (United States)

    ... this page: https://medlineplus.gov/libraries/indiana.html Libraries in Indiana To use the sharing features on ... please enable JavaScript. Evansville Deaconess Hospital Health Science Library 600 Mary Street Evansville, IN 47747 812-450- ...

  18. 77 FR 5843 - National Register of Historic Places; Notification of Pending Nominations and Related Actions

    Science.gov (United States)

    2012-02-06

    ... Sedgwick County Bitting Building, 107 N. Market St., Wichita, 12000046 KENTUCKY Boone County Roberts... removal has been made for the following resources: INDIANA Huntington County Snider, S.C., and George Mc...

  19. 78 FR 17157 - Approval of Air Quality Implementation Plans; Indiana; Disapproval of State Implementation Plan...

    Science.gov (United States)

    2013-03-20

    ... County, Indiana. This revision would remove the SO 2 emission limit for the blast furnace gas flare at... furnace gas that may be burned in the flare, or limit the frequency or duration of the flare's usage. The actual SO 2 emissions from the flare are determined by the total amount of gases it burns, and the...

  20. Archaeology in Indiana: The Science Today.

    Science.gov (United States)

    Jones, James R., III, Ed.; Johnson, Amy, Ed.; Bennett, Pamela J., Ed.

    1999-01-01

    This issue continues the Indiana Historical Bureau's collaboration with the Division of Historic Preservation and Archaeology, Indiana Department of Natural Resources. The articles include "The Science of Archaeology," chronicling the remarkable transformation of the science of archaeology to date; "Archaeology in Indiana," providing a brief…

  1. Huntington Disease in Asia

    Institute of Scientific and Technical Information of China (English)

    Miao Xu; Zhi-Ying Wu

    2015-01-01

    Objective:The objective was to review the major differences of Huntington disease (HD) in Asian population from those in the Caucasian population.Data Sources:Data cited in this review were obtained from PubMed database and China National Knowledge Infrastructure (CNKI) from 1994 to 2014.All the papers were written in English or Chinese languages,with the terms of Asia/Asian,HD,genotype,epidemiology,phenotype,and treatment used for the literature search.Study Selection:From the PubMed database,we included the articles and reviews which contained the HD patients' data from Asian countries.From the CNKI,we excluded the papers which were not original research.Due to the language's restrictions,those data published in other languages were not included.Results:In total,50 papers were cited in this review,authors of which were from the mainland of China,Japan,India,Thailand,Taiwan (China),Korea,and western countries.Conclusions:The lower epidemiology in Asians can be partly explained by the less cytosine-adenine-guanine repeats,different haplotypes,and CCG polymorphisms.For the physicians,atypical clinical profiles such as the initial symptom of ataxia,movement abnormalities of Parkinsonism,dystonia,or tics need to be paid more attention to and suggest gene testing if necessary.Moreover,some pathogenesis studies may help progress some new advanced treatments.The clinicians in Asian especially in China should promote the usage of genetic testing and put more effects in rehabilitation,palliative care,and offer comfort of patients and their families.The unified HD rating scale also needs to be popularized in Asia to assist in evaluating the progression of HD.

  2. Fuel alcohol opportunities for Indiana

    Energy Technology Data Exchange (ETDEWEB)

    Greenglass, Bert

    1980-08-01

    Prepared at the request of US Senator Birch Bayh, Chairman of the National Alcohol Fuels Commission, this study may be best utilized as a guidebook and resource manual to foster the development of a statewide fuel alcohol plan. It examines sectors in Indiana which will impact or be impacted upon by the fuel alcohol industry. The study describes fuel alcohol technologies that could be pertinent to Indiana and also looks closely at how such a fuel alcohol industry may affect the economic and policy development of the State. Finally, the study presents options for Indiana, taking into account the national context of the developing fuel alcohol industry which, unlike many others, will be highly decentralized and more under the control of the lifeblood of our society - the agricultural community.

  3. Mitochondrial dysfunction and Huntington disease

    Institute of Scientific and Technical Information of China (English)

    2006-01-01

    Huntington disease (HD) is a chronic autosomal-dominant neurodegenerative disease. The gene coding Huntingtin has been identified, but the pathogenic mechanisms of the disease are still not fully understood. This paper reviews the involvement of mitochondrial dysfunction in pathogenesis of HD.

  4. Is Huntington's disease a tauopathy?

    Science.gov (United States)

    Gratuze, Maud; Cisbani, Giulia; Cicchetti, Francesca; Planel, Emmanuel

    2016-04-01

    Tauopathies are a subclass of neurodegenerative diseases typified by the deposition of abnormal microtubule-associated tau protein within the cerebral tissue. Alzheimer's disease, progressive supranuclear palsy, chronic traumatic encephalopathy and some fronto-temporal dementias are examples of the extended family of tauopathies. In the last decades, intermittent reports of cerebral tau pathology in individuals afflicted with Huntington's disease-an autosomal dominant neurodegenerative disorder that manifests by severe motor, cognitive and psychiatric problems in adulthood-have also begun to surface. These observations remained anecdotal until recently when a series of publications brought forward compelling evidence that this monogenic disorder may, too, be a tauopathy. Collectively, these studies reported that: (i) patients with Huntington's disease present aggregated tau inclusions within various structures of the brain; (ii) tau haplotype influences the cognitive function of Huntington's disease patients; and (iii) that the genetic product of the disease, the mutant huntingtin protein, could alter tau splicing, phosphorylation, oligomerization and subcellular localization. Here, we review the past and current evidence in favour of the postulate that Huntington's disease is a new member of the family of tauopathies. © The Author (2016). Published by Oxford University Press on behalf of the Guarantors of Brain. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  5. Indiana Bat Project data

    Science.gov (United States)

    Erickson, Richard A.

    2016-01-01

    Our model is a full-annual-cycle population model {hostetler2015full} that tracks groups of bat surviving through four seasons: breeding season/summer, fall migration, non-breeding/winter, and spring migration. Our state variables are groups of bats that use a specific maternity colony/breeding site and hibernaculum/non-breeding site. Bats are also accounted for by life stages (juveniles/first-year breeders versus adults) and seasonal habitats (breeding versus non-breeding) during each year, This leads to four states variable (here depicted in vector notation): the population of juveniles during the non-breeding season, the population of adults during the non-breeding season, the population of juveniles during the breeding season, and the population of adults during the breeding season, Each vector's elements depict a specific migratory pathway, e.g., is comprised of elements, {non-breeding sites}, {breeding sites}The variables may be summed by either breeding site or non-breeding site to calculate the total population using a specific geographic location. Within our code, we account for this using an index column for breeding sites and an index column for non-breeding sides within the data table. Our choice of state variables caused the time step (i.e. \\(t\\)) to be 1 year. However, we recorded the population of each group during the breeding and non-breeding season as an artifact of our state-variable choice. We choose these state variables partially for their biological information and partially to simplify programming. We ran our simulation for 30 years because the USFWS currently issues Indiana Bat take permits for 30 years. Our model covers the range of the Indiana Bat, which is approximately the eastern half of the contiguous United States (Figure \\ref{fig:BatInput}). The boundaries of our range was based upon the United States boundary, the NatureServe Range map, and observations of the species. The maximum migration distance was 500-km, which was based

  6. Neurodegenerative disorders: Parkinson's disease and Huntington's disease

    Science.gov (United States)

    Hague, S; Klaffke, S; Bandmann, O

    2005-01-01

    Parkinson's disease and Huntington's disease are both model diseases. Parkinson's disease is the most common of several akinetic-rigid syndromes and Huntington's disease is only one of an ever growing number of trinucleotide repeat disorders. Molecular genetic studies and subsequent molecular biological studies have provided fascinating new insights into the pathogenesis of both disorders and there is now real hope for disease modifying treatment in the not too distant future for patients with Parkinson's disease or Huntington's disease. PMID:16024878

  7. Neurodegenerative disorders: Parkinson's disease and Huntington's disease.

    Science.gov (United States)

    Hague, S M; Klaffke, S; Bandmann, O

    2005-08-01

    Parkinson's disease and Huntington's disease are both model diseases. Parkinson's disease is the most common of several akinetic-rigid syndromes and Huntington's disease is only one of an ever growing number of trinucleotide repeat disorders. Molecular genetic studies and subsequent molecular biological studies have provided fascinating new insights into the pathogenesis of both disorders and there is now real hope for disease modifying treatment in the not too distant future for patients with Parkinson's disease or Huntington's disease.

  8. Huntington's disease presenting as amyotrophic lateral sclerosis.

    Science.gov (United States)

    Phukan, Julie; Ali, Elfatih; Pender, Niall P; Molloy, Fiona; Hennessy, Michael; Walsh, Ronan J; Hardiman, Orla

    2010-08-01

    We present the clinical, electrophysiological and molecular genetic findings of a 58-year-old male with genetically confirmed Huntington's disease (HD) and concurrent clinically definite ALS by El Escorial criteria. The patient presented with asymmetric upper limb amyotrophy and weakness, and subsequently developed chorea and cognitive change. Genetic testing confirmed the presence of expanded trinucleotide repeats in huntingtin, consistent with a diagnosis of Huntington's disease. This case confirms the rare coexistence of Huntington's disease and motor neuron degeneration.

  9. No More Indiana Jones Warehouses

    Science.gov (United States)

    Pannapacker, William

    2012-01-01

    In "Raiders of the Lost Ark," Indiana Jones--perhaps the last heroic professor to appear in a major Hollywood film--survives a series of adventures involving spiders, snakes, treacherous colleagues, and countless Nazis who are determined to recover the ark of the covenant for their "Fuhrer." Apparently the ark has mystical powers. Ultimately,…

  10. Huntington Disease: Molecular Diagnostics Approach.

    Science.gov (United States)

    Bastepe, Murat; Xin, Winnie

    2015-10-06

    Huntington disease (HD) is caused by expansion of a CAG trinucleotide repeat in the first exon of the Huntingtin (HTT) gene. Molecular testing of Huntington disease for diagnostic confirmation and disease prediction requires detection of the CAG repeat expansion. There are three main types of HD genetic testing: (1) diagnostic testing to confirm or rule out disease, (2) presymptomatic testing to determine whether an at-risk individual inherited the expanded allele, and (3) prenatal testing to determine whether the fetus has inherited the expanded allele. This unit includes protocols that describe the complementary use of polymerase chain reactions (PCR) and Southern blot hybridization to accurately measure the CAG trinucleotide repeat size and interpret the test results. In addition, an indirect linkage analysis that does not reveal the unwanted parental HD status in a prenatal testing will also be discussed.

  11. Cortical myoclonus in Huntington's disease.

    Science.gov (United States)

    Thompson, P D; Bhatia, K P; Brown, P; Davis, M B; Pires, M; Quinn, N P; Luthert, P; Honovar, M; O'Brien, M D; Marsden, C D

    1994-11-01

    We describe three patients with Huntington's disease, from two families, in whom myoclonus was the predominant clinical feature. The diagnosis was confirmed at autopsy in two cases and by DNA analysis in all three. These patients all presented before the age of 30 years and were the offspring of affected fathers. Neurophysiological studies documented generalised and multifocal action myoclonus of cortical origin that was strikingly stimulus sensitive, without enlargement of the cortical somatosensory evoked potential. The myoclonus improved with piracetam therapy in one patient and a combination of sodium valproate and clonazepam in the other two. Cortical reflex myoclonus is a rare but disabling component of the complex movement disorder of Huntington's disease, which may lead to substantial diagnostic difficulties.

  12. Molecular Imaging of Huntington's Disease.

    Science.gov (United States)

    Ciarmiello, Andrea; Giovacchini, Giampiero; Giovannini, Elisabetta; Lazzeri, Patrizia; Borsò, Elisa; Mannironi, Antonio; Mansi, Luigi

    2017-08-01

    The onset and the clinical progression of Huntington Disease (HD) is influenced by several events prompted by a genetic mutation that affects several organs tissues including different regions of the brain. In the last decades years, Positron Emission Tomography (PET) and Magnetic Resonance Imaging (MRI) helped to deepen the knowledge of neurodegenerative mechanisms that guide to clinical symptoms. Brain imaging with PET represents a tool to investigate the physiopathology occurring in the brain and it has been used to predict the age of onset of the disease and to evaluate the therapeutic efficacy of new drugs. This article reviews the contribution of PET and MRI in the research field on Huntington's disease, focusing in particular on some most relevant achievements that have helped recognize the molecular changes, the clinical symptoms and evolution of the disease. J. Cell. Physiol. 232: 1988-1993, 2017. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  13. Antipsychotic drugs in Huntington's disease.

    Science.gov (United States)

    Unti, E; Mazzucchi, S; Palermo, G; Bonuccelli, U; Ceravolo, R

    2017-03-01

    The aim of this review is to overview the pharmacological features of neuroleptics experienced in the treatment of Huntington's disease (HD) symptoms. Despite a large number of case reports, randomized controlled trials (RCT) and drug comparison studies are lacking. Areas covered: After evaluating current guidelines and clinical unmet needs we searched PubMed for the term 'Huntington's disease' cross referenced with the terms 'Antipsychotic drugs' 'Neuroleptic drugs' and single drug specific names. Expert commentary: In clinical practice antipsychotics represent the first choice in the management of chorea in the presence of psychiatric symptoms, when poor compliance is suspected or when there is an increased risk of adverse events due to tetrabenazine. Antipsychotics are considered valid strategies, with the second generation preferred to reduce extrapyramidal adverse events, however they may cause more metabolic side effects. In the future 'dopamine stabilizers', such as pridopidine, could replace antipsychotics modulating dopamine transmission.

  14. Distribution of Ehrlichia chaffeensis (Rickettsiales: Rickettsiaeceae) in Amblyomma americanum in southern Indiana and prevalence of E. chaffeensis--reactive antibodies in white-tailed deer in Indiana and Ohio in 1998.

    Science.gov (United States)

    Irving, R P; Pinger, R R; Vann, C N; Olesen, J B; Steiner, F E

    2000-07-01

    To continue monitoring the prevalence and distribution of Ehrlichia chaffeensis (Rickettsiales: Rickettsiaeceae) in southern Indiana, a total of 498 Amblyomma americanum (L.) ticks (262 adults and 292 nymphs) was collected from five southern Indiana counties during May and June 1998. Ticks were pooled and examined for the presence of E. chaffeensis using nested polymerase chain reaction and primers specific for the 16S rRNA gene of E. chaffeensis. The average minimum infection rate for adult ticks collected in 1998 was 3.8% (ranging from 0 to 7.7% in various counties) as compared with previous average minimum infection rates of 1.6% in 1995 and 4.9% in 1997. None of the pools of A. americanum nymphs tested positive. In addition, blood samples were collected from 325 white-tailed deer taken in Indiana and 327 taken in Ohio in November 1998. Serum samples were tested for the presence of E. chaffeensis-like organisms reactive to antibodies using an indirect immunofluorescence assay (IFA). Antibodies were found in deer from six Indiana counties where infection rates ranged from 42.6 to 66.7% and in four Ohio countries where infection rates ranged from 4.4 to 25%. The results of this study reconfirm that E. chaffeensis is well established in southern Indiana and also provide the first evidence of E. chaffeensis-like organisms infecting white-tailed deer in Ohio, suggesting the need to survey Ohio ticks for the presence of Ehrlichia.

  15. Neuropsychiatric Burden in Huntington's Disease.

    Science.gov (United States)

    Paoli, Ricardo Augusto; Botturi, Andrea; Ciammola, Andrea; Silani, Vincenzo; Prunas, Cecilia; Lucchiari, Claudio; Zugno, Elisa; Caletti, Elisabetta

    2017-06-16

    Huntington's disease is a disorder that results in motor, cognitive, and psychiatric problems. The symptoms often take different forms and the presence of disturbances of the psychic sphere reduces patients' autonomy and quality of life, also impacting patients' social life. It is estimated that a prevalence between 33% and 76% of the main psychiatric syndromes may arise in different phases of the disease, often in atypical form, even 20 years before the onset of chorea and dementia. We present a narrative review of the literature describing the main psychopathological patterns that may be found in Huntington's disease, searching for a related article in the main database sources (Medline, ISI Web of Knowledge, Scopus, and Medscape). Psychiatric conditions were classified into two main categories: affective and nonaffective disorders/symptoms; and anxiety and neuropsychiatric features such as apathy and irritability. Though the literature is extensive, it is not always convergent, probably due to the high heterogeneity of methods used. We summarize main papers for pathology and sample size, in order to present a synoptic vision of the argument. Since the association between Huntington's disease and psychiatric symptoms was demonstrated, we argue that the prevalent and more invalidating psychiatric components should be recognized as early as possible during the disease course in order to best address psychopharmacological therapy, improve quality of life, and also reduce burden on caregivers.

  16. "Salatoimikud" Indiana Jonesi moodi / Inna-Katrin Hein

    Index Scriptorium Estoniae

    Hein, Inna-Katrin

    2008-01-01

    Steven Spielbergi neljas Indiana Jones'i film Harrison Fordiga nimiosas "Indiana Jones ja kristallpealuu kuningriik" ("Indiana Jones and the Kingdom of the Crystal Skull") : Ameerika Ühendriigid 2008

  17. "Salatoimikud" Indiana Jonesi moodi / Inna-Katrin Hein

    Index Scriptorium Estoniae

    Hein, Inna-Katrin

    2008-01-01

    Steven Spielbergi neljas Indiana Jones'i film Harrison Fordiga nimiosas "Indiana Jones ja kristallpealuu kuningriik" ("Indiana Jones and the Kingdom of the Crystal Skull") : Ameerika Ühendriigid 2008

  18. Huntington's disease: review and anesthetic case management.

    OpenAIRE

    Cangemi, C. F.; Miller, R. J.

    1998-01-01

    Huntington's disease is a dominantly inherited progressive autosomal disease that affects the basal ganglia. Symptoms appear later in life and manifest as progressive mental deterioration and involuntary choreiform movements. Patients with Huntington's disease develop a progressive but variable dementia. Dysphagia, the most significant related motor symptom, hinders nutrition intake and places the patient at risk for aspiration. The combination of involuntary choreoathetoid movements, depress...

  19. Drug-induced hyperthermia in Huntington's disease

    NARCIS (Netherlands)

    Gaasbeek, D; Naarding, Paul; Stor, T; Kremer, H P H

    Until now, only three patients with Huntington's disease (HD) and a neuroleptic malignant syndrome (NMS) have been reported in the literature. We describe four cases with advanced stage Huntington's disease who within a period of one year developed drug-induced hyperthermia, either the neuroleptic

  20. Drug-induced hyperthermia in Huntington's disease.

    NARCIS (Netherlands)

    Gaasbeek, D.; Naarding, P.; Stor, T.; Kremer, H.P.H.

    2004-01-01

    Until now, only three patients with Huntington's disease (HD) and a neuroleptic malignant syndrome (NMS) have been reported in the literature. We describe four cases with advanced stage Huntington's disease who within a period of one year developed drug-induced hyperthermia, either the neuroleptic

  1. Drug-induced hyperthermia in Huntington's disease.

    NARCIS (Netherlands)

    Gaasbeek, D.; Naarding, P.; Stor, T.; Kremer, H.P.H.

    2004-01-01

    Until now, only three patients with Huntington's disease (HD) and a neuroleptic malignant syndrome (NMS) have been reported in the literature. We describe four cases with advanced stage Huntington's disease who within a period of one year developed drug-induced hyperthermia, either the neuroleptic m

  2. Apathy is not depression in Huntington's disease

    NARCIS (Netherlands)

    Naarding, Paul; Janzing, Joost G E; Eling, Paul; van der Werf, Sieberen; Kremer, Berry

    2009-01-01

    Apathy and depression are common neuropsychiatric features of Huntington's disease. The authors studied a group of 34 Huntington's disease patients. In addition to the conventional classification according to DSM-IV criteria of depression, emphasis was put on a dimensional approach using scores on

  3. Huntington's disease: clinical characteristics, pathogenesis and therapies.

    Science.gov (United States)

    Nakamura, Ken; Aminoff, Michael J

    2007-02-01

    Huntington's disease is a devastating disorder with no known cure. The disease results from an expanded sequence of CAG repeats in the huntingtin gene and leads to a movement disorder with associated cognitive and systemic deficits. Huntington's disease is diagnosed by genetic testing and disease progression can be followed with a variety of imaging modalities. The accumulation of aggregated huntingtin with associated striatal degeneration is evident at autopsy. The pathophysiology of Huntington's disease remains unknown, although protein aggregation, excitotoxicity, deficits in energy metabolism, transcriptional dysregulation and apoptosis may all be involved. Current pharmacologic therapy for Huntington's disease is limited and exclusively symptomatic. However, the disease is being heavily researched, and a wide range of disease-modifying therapies is currently under development. The efficacy of these therapies is being evaluated in transgenic models of Huntington's disease and in preliminary clinical trials.

  4. Study of application of ERTS-A imagery to fracture-related mine safety hazards in the coal mining industry. [Indiana

    Science.gov (United States)

    Wier, C. E.; Wobber, F. J. (Principal Investigator); Russell, O. R.; Amato, R. V.; Leshendok, T.

    1973-01-01

    The author has identified the following significant results. The Mined Land Inventory map of Pike, Gibson, and Warrick Counties, Indiana, prepared from ERTS-1 imagery, was included in the 1973 Annual Report of the President's Council on Environmental Quality as an example of ERTS applications to mined lands. Increasing numbers of inquiries have been received from coal producing states and coal companies interested in the Indiana Program.

  5. 78 FR 8018 - Establishment of the Indiana Uplands Viticultural Area and Modification of the Ohio River Valley...

    Science.gov (United States)

    2013-02-05

    ...) generally easterly to the mouth of French Creek in Franklin Township, Floyd County, Indiana (Louisville map..., approximately 0.3 mile south of the White River; then (6) Proceed easterly along the meandering 200-meter... easterly then southwesterly along the meandering 200- meter contour line, crossing to the Bedford map,...

  6. 75 FR 48359 - Endangered and Threatened Wildlife and Plants; Indiana Bat; Notice of Intent To Prepare an...

    Science.gov (United States)

    2010-08-10

    ... Indiana Bat (Myotis sodalis) at a wind power project in Adair, Sullivan, and Putnam Counties, Missouri... Endangered and Threatened Wildlife and Plants on March 11, 1967 (32 FR 4001). It is currently listed as an... dying trees or living trees with broken and flaking bark. There are no known hibernacula in the...

  7. Huntington's Disease: An Immune Perspective

    Directory of Open Access Journals (Sweden)

    Annapurna Nayak

    2011-01-01

    Full Text Available Huntington's disease (HD is a progressive neurodegenerative disorder that is caused by abnormal expansion of CAG trinucleotide repeats. Neuroinflammation is a typical feature of most neurodegenerative diseases that leads to an array of pathological changes within the affected areas in the brain. The neurodegeneration in HD is also caused by aberrant immune response in the presence of aggregated mutant huntingtin protein. The effects of immune activation in HD nervous system are a relatively unexplored area of research. This paper summarises immunological features associated with development and progression of HD.

  8. Huntington disease: pathogenesis and treatment.

    Science.gov (United States)

    Dayalu, Praveen; Albin, Roger L

    2015-02-01

    Huntington disease (HD) is an autosomal dominant inherited neurodegenerative disease characterized by progressive motor, behavioral, and cognitive decline, culminating in death. It is caused by an expanded CAG repeat in the huntingtin gene. Even years before symptoms become overt, mutation carriers show subtle but progressive striatal and cerebral white matter atrophy by volumetric MRI. Although there is currently no direct treatment of HD, management options are available for several symptoms. A better understanding of HD pathogenesis, and more sophisticated clinical trials using newer biomarkers, may lead to meaningful treatments. This article reviews the current knowledge of HD pathogenesis and treatment.

  9. Psychiatric symptoms and CAG expansion in Huntington`s disease

    Energy Technology Data Exchange (ETDEWEB)

    Weber, M.W.; Schmid, W.; Spiegel, R. [Univ. of Zuerich (Switzerland)

    1996-02-16

    The mutation responsible for Huntington`s disease (HD) is an elongated CAG repeat in the coding region of the IT15 gene. A PCR-based test with high sensitivity and accuracy is now available to identify asymptomatic gene carriers and patients. An inverse correlation between CAG copy number and age at disease onset has been found in a large number of affected individuals. The influence of the CAG repeat expansion on other phenotypic manifestations, especially specific psychiatric symptoms has not been studied intensively. In order to elucidate this situation we investigated the relation between CAG copy number and distinct psychiatric phenotypes found in 79 HD-patients. None of the four differentiated categories (personality change, psychosis, depression, and nonspecific alterations) showed significant differences in respect to size of the CAG expansion. In addition, no influence of individual sex on psychiatric presentation could be found. On the other hand in patients with personality changes maternal transmission was significantly more frequent compared with all other groups. Therefore we suggest that clinical severity of psychiatric features in HD is not directly dependent on the size of the dynamic mutation involved. The complex pathogenetic mechanisms leading to psychiatric alterations are still unknown and thus genotyping does not provide information about expected psychiatric symptoms in HD gene carriers. 40 refs., 1 fig., 2 tabs.

  10. 77 FR 16315 - Indiana Disaster #IN-00041

    Science.gov (United States)

    2012-03-20

    ... ADMINISTRATION Indiana Disaster IN-00041 AGENCY: U.S. Small Business Administration. ACTION: Notice. SUMMARY: This is a Notice of the Presidential declaration of a major disaster for the State of Indiana (FEMA... Disaster Assistance, U.S. Small Business Administration, 409 3rd Street SW., Suite 6050, Washington,...

  11. 76 FR 59176 - Indiana Disaster #IN-00036

    Science.gov (United States)

    2011-09-23

    ... ADMINISTRATION Indiana Disaster IN-00036 AGENCY: U.S. Small Business Administration. ACTION: Notice. SUMMARY: This is a notice of an Administrative declaration of a disaster for the State of Indiana dated 09/12... 76155. FOR FURTHER INFORMATION CONTACT: A. Escobar, Office of Disaster Assistance, U.S. Small...

  12. 77 FR 41680 - Indiana Regulatory Program

    Science.gov (United States)

    2012-07-16

    ... Survey; and 312 IAC 25-6-95 Underground Mining, Explosives, Publication of Blasting Schedule Indiana... surface blasting regulations would apply. Indiana also removed the requirement to submit a blast design... provisions, periods of liability, performance bond release, revegetation standards, underground...

  13. Survey of Indiana Correctional Institution Libraries.

    Science.gov (United States)

    Indiana State Library, Indianapolis.

    This study of the adequacy of library facilities in Indiana's penal institutions was undertaken at the request of a state senate committee studying the Indiana Department of Correction. The penal libraries are judged to be inadequate and their major problems with personnel, materials, services, and physical facilities are listed. Also given are…

  14. Indiana residents' perceptions of woodland management

    Science.gov (United States)

    Daniel J. Witter; Shannon M. Amberg; David J. Case; Phillip T. Seng

    2013-01-01

    A 2009 telephone survey of 1,402 Indiana adults was conducted to assess opinions regarding woodland management. Forty-eight percent said they were "very concerned" about the health and productivity of Indiana's woodlands, and 45 percent, "somewhat concerned." Almost half (47 percent) thought that the state's woodlands are held in about...

  15. [Molecular therapeutic strategies for Huntington's disease].

    Science.gov (United States)

    Milewski, Michał; Hoffman-Zacharska, Dorota; Ball, Jerzy

    2015-01-01

    Huntington's disease is a progressive neurodegenerative disorder of genetic origin that still lacks an effective treatment. Recently, a number of new attempts have been undertaken to develop a successful molecular therapy for this incurable condition. The novel approaches employ, among others, some new methods to selectively silence the mutated gene or to neutralize its toxic protein product. This paper reviews all major strategies that are currently considered for molecular therapy of Huntington's disease while discussing their potential effectiveness regarding the treatment of both the Huntington's disease and a large group of related neurodegenerative disorders associated with abnormal protein aggregation.

  16. Genetic modifiers of Huntington's disease.

    Science.gov (United States)

    Gusella, James F; MacDonald, Marcy E; Lee, Jong-Min

    2014-09-15

    Huntington's disease (HD) is a devastating neurodegenerative disorder that directly affects more than 1 in 10,000 persons in Western societies but, as a family disorder with a long, costly, debilitating course, it has an indirect impact on a far greater proportion of the population. Although some palliative treatments are used, no effective treatment exists for preventing clinical onset of the disorder or for delaying its inevitable progression toward premature death, approximately 15 years after diagnosis. Huntington's disease involves a movement disorder characterized by chorea, as well as a variety of psychiatric disturbances and intellectual decline, with a gradual loss of independence. A dire need exists for effective HD therapies to alleviate the suffering and costs to the individual, family, and health care system. In past decades, genetics, the study of DNA sequence variation and its consequences, provided the tools to map the HD gene to chromosome 4 and ultimately to identify its mutation as an expanded CAG trinucleotide repeat in the coding sequence of a large protein, dubbed huntingtin. Now, advances in genetic technology offer an unbiased route to the identification of genetic factors that are disease-modifying agents in human patients. Such genetic modifiers are expected to highlight processes capable of altering the course of HD and therefore to provide new, human-validated targets for traditional drug development, with the goal of developing rational treatments to delay or prevent onset of HD clinical signs.

  17. Huntington's disease: review and anesthetic case management.

    Science.gov (United States)

    Cangemi, C F; Miller, R J

    1998-01-01

    Huntington's disease is a dominantly inherited progressive autosomal disease that affects the basal ganglia. Symptoms appear later in life and manifest as progressive mental deterioration and involuntary choreiform movements. Patients with Huntington's disease develop a progressive but variable dementia. Dysphagia, the most significant related motor symptom, hinders nutrition intake and places the patient at risk for aspiration. The combination of involuntary choreoathetoid movements, depression, and apathy leads to cachexia. Factors of considerable concern to the anesthesiologist who treats patients with Huntington's disease may include how to treat frail elderly people incapable of cooperation, how to treat patients suffering from malnourishment, and how to treat patients with an increased risk for aspiration or exaggerated responses to sodium thiopental and succinylcholine. The successful anesthetic management of a 65-yr-old woman with Huntington's disease who presented for full-mouth extractions is described.

  18. Huntington's Disease: Speech, Language and Swallowing

    Science.gov (United States)

    ... Disease Society of America Huntington's Disease Youth Organization Movement Disorder Society National Institute of Neurological Disorders and Stroke Typical Speech and Language Development Learning More Than One Language Adult Speech and Language Child Speech and Language Swallowing ...

  19. Impaired mitochondrial trafficking in Huntington's disease

    OpenAIRE

    Li, Xiao-Jiang; Orr, Adam L.; Li, Shihua

    2009-01-01

    Abstract Impaired mitochondrial function has been well documented in Huntington?s disease. Mutant huntingtin is found to affect mitochondria via various mechanisms including the dysregulation of gene transcription and impairment of mitochondrial function or trafficking. The lengthy and highly branched neuronal processes constitute complex neural networks in which there is a large demand for mitochondria-generated energy. Thus, the impaired mitochondria trafficking in neuronal cells...

  20. Indiana continent catheterizable urinary reservoir.

    Science.gov (United States)

    Castillo, O A; Aranguren, G; Campos-Juanatey, F

    2014-01-01

    Radical pelvic surgery requires continent or incontinent urinary diversion. There are many techniques, but the orthotopic neobladder is the most used. A continent catheterizable urinary reservoir is sometimes a good alternative when this derivation is not possible or not indicated. This paper has aimed to present our experience with the Indiana pouch continent urinary reservoir. The series is made up of 85 patients, 66 women and 19 men, with a mean age of 56 years (31-77 years). Variables analyzed were operating time, estimated blood loss, transfusion rate, hospital stay and peri-operatory complications. The main indication in 49 cases was resolution of complications related to the treatment of cervical cancer. Average operation time was 110.5 minutes (range 80-130 minutes). Mean blood loss was 450 cc (100-1000 cc). Immediate postoperative complications, all of which were treated medically, occurred in 16 patients (18.85%). One patient suffered anastomotic leakage. Hospital stay was 19 days (range 5-60 days) and there was no mortality in the series. Late complications occurred in 26 patients (32%), these being ureteral anastomotic stenosis in 11 cases, cutaneous stoma stenosis in 9 cases and reservoir stones in 6 cases. The Indiana continent catheterizable urinary reservoir is a valid option for the treatment of both urological and gynecological malignancies as well as for the management of pelvic morbidity related to the treatment of pelvic cancers. Copyright © 2013 AEU. Published by Elsevier Espana. All rights reserved.

  1. Clinical presentation of juvenile Huntington disease

    Directory of Open Access Journals (Sweden)

    Ruocco Heloísa H.

    2006-01-01

    Full Text Available OBJECTIVE: To describe the clinical presentation a group of patients with juvenile onset of Huntington disease. METHOD: All patients were interviewed following a structured clinical questioner. Patients were genotyped for the trinucleotide cytosine-adenine-guanine (CAG repeat in the Huntington Disease gene. High resolution brain MRI was performed in all patients. RESULTS: We identified 4 patients with juvenile onset of disease among 50 patients with Huntington disease followed prospectively in our Neurogenetics clinic. Age at onset varied from 3 to 13 years, there were 2 boys, and 3 patients had a paternal inheritance of the disease. Expanded Huntington disease allele sizes varied from 41 to 69 trinucleotide repeats. The early onset patients presented with rigidity, bradykinesia, dystonia, dysarthria, seizures and ataxia. MRI showed severe volume loss of caudate and putamen nuclei (p=0.001 and reduced cerebral and cerebellum volumes (p=0.01. CONCLUSION: 8% of Huntington disease patients seen in our clinic had juvenile onset of the disease. They did not present with typical chorea as seen in adult onset Huntington disease. There was a predominance of rigidity and bradykinesia. Two other important clinical features were seizures and ataxia, which related with the imaging findings of early cortical atrophy and cerebellum volume loss.

  2. 40 CFR 81.315 - Indiana.

    Science.gov (United States)

    2010-07-01

    ... County X LaPorte County X Marion County X Porter County: An area bound on the north by Lake Michigan, on the west by the Lake-Porter County line, on the south by I-80 and 90 and on the east by the LaPorte-Porter County line ..... X The remainder of Porter County...... X Vigo County X Warrick County 1 X Wayne...

  3. Ethical issues and Huntington's disease.

    Science.gov (United States)

    Kromberg, Jennifer G R; Wessels, Tina-Marié

    2013-10-11

    The practice of genetic counselling gives rise to many ethical dilemmas, and counsellors need to be familiar with the principles of biomedical ethics. The primary principles include respect for autonomy, beneficence, non-maleficence and justice. A case of identical twins at 50% risk for Huntington's disease, in which only one twin sought predictive testing for this dominantly inherited disease, created several ethical dilemmas. Another case where predictive testing was carried out on two young children, at high risk, by a laboratory at the request of an adoption agency and a doctor, with a view to giving information to the foster parents, also posed many ethical conundrums for the counsellor. The ethical issues that arose in these cases are discussed in this paper. 

  4. Cholesterol metabolism in Huntington disease.

    Science.gov (United States)

    Karasinska, Joanna M; Hayden, Michael R

    2011-09-06

    The CNS is rich in cholesterol, which is essential for neuronal development and survival, synapse maturation, and optimal synaptic activity. Alterations in brain cholesterol homeostasis are linked to neurodegeneration. Studies have demonstrated that Huntington disease (HD), a progressive and fatal neurodegenerative disorder resulting from polyglutamine expansion in the huntingtin protein, is associated with changes in cellular cholesterol metabolism. Emerging evidence from human and animal studies indicates that attenuated brain sterol synthesis and accumulation of cholesterol in neuronal membranes represent two distinct mechanisms occurring in the presence of mutant huntingtin that influence neuronal survival. Increased knowledge of how changes in intraneuronal cholesterol metabolism influence the pathogenesis of HD will provide insights into the potential application of brain cholesterol regulation as a therapeutic strategy for this devastating disease.

  5. Juvenile Huntington disease in Argentina.

    Science.gov (United States)

    Gatto, Emilia Mabel; Parisi, Virginia; Etcheverry, José Luis; Sanguinetti, Ana; Cordi, Lorena; Binelli, Adrian; Persi, Gabriel; Squitieri, Ferdinando

    2016-01-01

    We analyzed demographic, clinical and genetic characteristics of juvenile Huntington disease (JHD) and it frequency in an Argentinean cohort. Age at onset was defined as the age at which behavioral, cognitive, psychiatric or motor abnormalities suggestive of JHD were first reported. Clinical and genetic data were similar to other international series, however, in this context we identified the highest JHD frequency reported so far (19.72%; 14/71). Age at onset of JHD is challenging and still under discussion. Our findings reinforce the hypothesis that clinical manifestations, other than the typical movement disorder, may anticipate age at onset of even many years. Analyses of JHD cohorts are required to explore it frequency in populations with different backgrounds to avoid an underestimation of this rare phenotype. Moreover, data from selected populations may open new pathways in therapeutic approaches and may explain new potential correlations between HD presentations and environmental or biological factors.

  6. Language impairment in Huntington's disease.

    Science.gov (United States)

    Azambuja, Mariana Jardim; Radanovic, Marcia; Haddad, Mônica Santoro; Adda, Carla Cristina; Barbosa, Egberto Reis; Mansur, Letícia Lessa

    2012-06-01

    Language alterations in Huntington's disease (HD) are reported, but their nature and correlation with other cognitive impairments are still under investigation. This study aimed to characterize the language disturbances in HD and to correlate them to motor and cognitive aspects of the disease. We studied 23 HD patients and 23 controls, matched for age and schooling, using the Boston Diagnostic Aphasia Examination, Boston Naming Test, the Token Test, Animal fluency, Action fluency, FAS-COWA, the Symbol Digit Modalities Test, the Stroop Test and the Hooper Visual Organization Test (HVOT). HD patients performed poorer in verbal fluency (poral comprehension (preading comprehension (p=0.034) and narrative writing (p<0.0001). There was a moderate correlation between the Expressive Component and Language Competency Indexes and the HVOT (r=0.519, p=0.011 and r=0.450, p=0.031, respectively). Language alterations in HD seem to reflect a derangement in both frontostriatal and frontotemporal regions.

  7. Huntington's disease: a clinical review

    Directory of Open Access Journals (Sweden)

    Roos Raymund AC

    2010-12-01

    Full Text Available Abstract Huntington disease (HD is a rare neurodegenerative disorder of the central nervous system characterized by unwanted choreatic movements, behavioral and psychiatric disturbances and dementia. Prevalence in the Caucasian population is estimated at 1/10,000-1/20,000. Mean age at onset of symptoms is 30-50 years. In some cases symptoms start before the age of 20 years with behavior disturbances and learning difficulties at school (Juvenile Huntington's disease; JHD. The classic sign is chorea that gradually spreads to all muscles. All psychomotor processes become severely retarded. Patients experience psychiatric symptoms and cognitive decline. HD is an autosomal dominant inherited disease caused by an elongated CAG repeat (36 repeats or more on the short arm of chromosome 4p16.3 in the Huntingtine gene. The longer the CAG repeat, the earlier the onset of disease. In cases of JHD the repeat often exceeds 55. Diagnosis is based on clinical symptoms and signs in an individual with a parent with proven HD, and is confirmed by DNA determination. Pre-manifest diagnosis should only be performed by multidisciplinary teams in healthy at-risk adult individuals who want to know whether they carry the mutation or not. Differential diagnoses include other causes of chorea including general internal disorders or iatrogenic disorders. Phenocopies (clinically diagnosed cases of HD without the genetic mutation are observed. Prenatal diagnosis is possible by chorionic villus sampling or amniocentesis. Preimplantation diagnosis with in vitro fertilization is offered in several countries. There is no cure. Management should be multidisciplinary and is based on treating symptoms with a view to improving quality of life. Chorea is treated with dopamine receptor blocking or depleting agents. Medication and non-medical care for depression and aggressive behavior may be required. The progression of the disease leads to a complete dependency in daily life, which

  8. Discrepancies in reporting the CAG repeat lengths for Huntington's disease

    DEFF Research Database (Denmark)

    Quarrell, Oliver W; Handley, Olivia; O'Donovan, Kirsty

    2011-01-01

    Huntington's disease results from a CAG repeat expansion within the Huntingtin gene; this is measured routinely in diagnostic laboratories. The European Huntington's Disease Network REGISTRY project centrally measures CAG repeat lengths on fresh samples; these were compared with the original...

  9. Legislative Districts - REDISTRICTING_INDIANA_SENATE_2011_IED: Redistricted Legislative Boundaries for the Indiana Senate, Established in 2011 (Indiana Election Division, Polygon Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — This data set shows the results for the redistricting of legislative boundaries for the Indiana Senate for 2011 through 2021. Republicans who control the Indiana...

  10. Silencing Huntington's chorea: Is RNA Interference a Potential Cure?

    OpenAIRE

    Metz, Gerlinde A.; Whishaw, Ian Q.; Afra Foroud; Nafisa M Jadavji

    2006-01-01

    In 1872, George Huntington described Huntington's disease as characterized by motor, cognitive and psychiatric impairments. Huntington's disease is a dominant and autosomal mutation on chromosome 4 featuring the insertion of numerous CAG repeats. CAG codes for the amino acid, glutmanine that forms part of the Huntingtin protein (htt). Excess glutamine attachments make htt prone to accumulate in neurons. Three genes can be considered when developing therapies for Huntington's disease. They inc...

  11. Exercise effects in Huntington disease.

    Science.gov (United States)

    Frese, Sebastian; Petersen, Jens A; Ligon-Auer, Maria; Mueller, Sandro Manuel; Mihaylova, Violeta; Gehrig, Saskia M; Kana, Veronika; Rushing, Elisabeth J; Unterburger, Evelyn; Kägi, Georg; Burgunder, Jean-Marc; Toigo, Marco; Jung, Hans H

    2017-01-01

    Huntington disease (HD) is a relentlessly progressive neurodegenerative disorder with symptoms across a wide range of neurological domains, including cognitive and motor dysfunction. There is still no causative treatment for HD but environmental factors such as passive lifestyle may modulate disease onset and progression. In humans, multidisciplinary rehabilitation has a positive impact on cognitive functions. However, a specific role for exercise as a component of an environmental enrichment effect has been difficult to demonstrate. We aimed at investigating whether endurance training (ET) stabilizes the progression of motor and cognitive dysfunction and ameliorates cardiovascular function in HD patients. Twelve male HD patients (mean ± SD, 54.8 ± 7.1 years) and twelve male controls (49.1 ± 6.8 years) completed 26 weeks of endurance training. Before and after the training intervention, clinical assessments, exercise physiological tests, and a body composition measurement were conducted and a muscle biopsy was taken from M. vastus lateralis. To examine the natural course of the disease, HD patients were additionally assessed 6 months prior to ET. During the ET period, there was a motor deficit stabilization as indicated by the Unified Huntington's Disease Rating Scale motor section score in HD patients (baseline: 18.6 ± 9.2, pre-training: 26.0 ± 13.7, post-training: 26.8 ± 16.4). Peak oxygen uptake ([Formula: see text]) significantly increased in HD patients (∆[Formula: see text] = +0.33 ± 0.28 l) and controls (∆[Formula: see text] = +0.29 ± 0.41 l). No adverse effects of the training intervention were reported. Our results confirm that HD patients are amenable to a specific exercise-induced therapeutic strategy indicated by an increased cardiovascular function and a stabilization of motor function.

  12. Biochemical aspects of Huntington's chorea.

    Science.gov (United States)

    Caraceni, T; Calderini, G; Consolazione, A; Riva, E; Algeri, S; Girotti, F; Spreafico, R; Branciforti, A; Dall'olio, A; Morselli, P L

    1977-01-01

    Fifteen patients affected by Huntington's chorea were divided into two groups, 'slow' and 'fast', according to IQ scores on the Wechsler-Bellevue scale, and scores on some motor performance tests. A possible correlation was looked for between some biochemical data (cerebrospinal fluid (CSF), homovanillic acid (HVA), and 5-hydroxyindolacetic acid (5HIAA) levels, plasma dopamine-beta-hydroxylase (DBH), dopamine (DA) uptake by platelets), and clinical data (duration of illness, severity of symptoms, age of patients, IQ scores, 'slow' and 'fast' groups). The CSF, HVA, and 5HIAA levels were found to be significantly lowered in comparison with normal controls. DBH activity and DA uptake by platelets did not differ significantly from normal subjects. Treatment with haloperidol in all patients and with dipropylacetic acid in three patients did not appear to modify the CSF, HVA, and 5HIAA concentrations, the plasma DBH activity, or the DA uptake. There were no significant differences in the CSF, HVA, and 5HIAA contents between the two groups of patients, and there was no correlation between biochemical data and clinical features. PMID:143508

  13. Protein oxidation in Huntington disease.

    Science.gov (United States)

    Sorolla, M Alba; Rodríguez-Colman, María José; Vall-llaura, Núria; Tamarit, Jordi; Ros, Joaquim; Cabiscol, Elisa

    2012-01-01

    Huntington disease (HD) is an inherited neurodegenerative disorder caused by expansion of CAG repeats in the huntingtin gene, affecting initially the striatum and progressively the cortex. Oxidative stress, and consequent protein oxidation, has been described as important to disease progression. This review focuses on recent advances in the field, with a particular emphasis on the identified target proteins and the role that their oxidation has or might have in the pathophysiology of HD. Oxidation and the resulting inactivation and/or degradation of important proteins can explain the impairment of several metabolic pathways in HD. Oxidation of enzymes involved in ATP synthesis can account for the energy deficiency observed. Impairment of protein folding and degradation can be due to oxidation of several heat shock proteins and Valosin-containing protein. Oxidation of two enzymes involved in the vitamin B6 metabolism could result in decreased availability of pyridoxal phosphate, which is a necessary cofactor in transaminations, the kynurenine pathway and the synthesis of glutathione, GABA, dopamine and serotonin, all of which have a key role in HD pathology. In addition, protein oxidation often contributes to oxidative stress, aggravating the molecular damage inside the cell. Copyright © 2012 International Union of Biochemistry and Molecular Biology, Inc.

  14. Error processing in Huntington's disease.

    Directory of Open Access Journals (Sweden)

    Christian Beste

    Full Text Available BACKGROUND: Huntington's disease (HD is a genetic disorder expressed by a degeneration of the basal ganglia inter alia accompanied with dopaminergic alterations. These dopaminergic alterations are related to genetic factors i.e., CAG-repeat expansion. The error (related negativity (Ne/ERN, a cognitive event-related potential related to performance monitoring, is generated in the anterior cingulate cortex (ACC and supposed to depend on the dopaminergic system. The Ne is reduced in Parkinson's Disease (PD. Due to a dopaminergic deficit in HD, a reduction of the Ne is also likely. Furthermore it is assumed that movement dysfunction emerges as a consequence of dysfunctional error-feedback processing. Since dopaminergic alterations are related to the CAG-repeat, a Ne reduction may furthermore also be related to the genetic disease load. METHODOLOGY/PRINCIPLE FINDINGS: We assessed the error negativity (Ne in a speeded reaction task under consideration of the underlying genetic abnormalities. HD patients showed a specific reduction in the Ne, which suggests impaired error processing in these patients. Furthermore, the Ne was closely related to CAG-repeat expansion. CONCLUSIONS/SIGNIFICANCE: The reduction of the Ne is likely to be an effect of the dopaminergic pathology. The result resembles findings in Parkinson's Disease. As such the Ne might be a measure for the integrity of striatal dopaminergic output function. The relation to the CAG-repeat expansion indicates that the Ne could serve as a gene-associated "cognitive" biomarker in HD.

  15. Predictive testing for Huntington's disease.

    Science.gov (United States)

    Tibben, Aad

    2007-04-30

    Worldwide, predictive testing for Huntington's disease has become an accepted clinical application that has allowed many individuals from HD-families to proceed with their life without the uncertainty of being at risk. International guidelines have extensively contributed to establishing counselling programmes of high quality, and have served as a model for other genetic disorders. Psychological follow-up studies have increased the insight into the far-reaching impact of test results for all individuals involved. Although the guidelines have served as a useful frame of reference, clinical experience has shown the importance of a case-by-case approach to do justice to the specific needs of the individual test candidate. Issues such as ambiguous test results, lack of awareness in a test candidate of early signs of the disease, non-compliance to the test protocol, or the test candidate's need for information on the relationship between age at onset and CAG-repeat require careful consideration. Receiving a test result is only one of the transition points in the life of an individual at risk; such result needs to be valued from a life-cycle perspective.

  16. Huntington's Disease and Striatal Signaling.

    Science.gov (United States)

    Roze, Emmanuel; Cahill, Emma; Martin, Elodie; Bonnet, Cecilia; Vanhoutte, Peter; Betuing, Sandrine; Caboche, Jocelyne

    2011-01-01

    Huntington's Disease (HD) is the most frequent neurodegenerative disease caused by an expansion of polyglutamines (CAG). The main clinical manifestations of HD are chorea, cognitive impairment, and psychiatric disorders. The transmission of HD is autosomal dominant with a complete penetrance. HD has a single genetic cause, a well-defined neuropathology, and informative pre-manifest genetic testing of the disease is available. Striatal atrophy begins as early as 15 years before disease onset and continues throughout the period of manifest illness. Therefore, patients could theoretically benefit from therapy at early stages of the disease. One important characteristic of HD is the striatal vulnerability to neurodegeneration, despite similar expression of the protein in other brain areas. Aggregation of the mutated Huntingtin (HTT), impaired axonal transport, excitotoxicity, transcriptional dysregulation as well as mitochondrial dysfunction, and energy deficits, are all part of the cellular events that underlie neuronal dysfunction and striatal death. Among these non-exclusive mechanisms, an alteration of striatal signaling is thought to orchestrate the downstream events involved in the cascade of striatal dysfunction.

  17. Therapeutic advances in Huntington's Disease.

    Science.gov (United States)

    Shannon, Kathleen M; Fraint, Avram

    2015-09-15

    Huntington's disease is a rare hereditary degenerative disease with a wide variety of symptoms that encompass movement, cognition, and behavior. The genetic mutation that causes the disease has been known for more than 20 y, and animal models have illuminated a host of intracellular derangements that occur downstream of protein translation. A number of clinical trials targeting these metabolic consequences have failed to produce a single effective therapy, although clinical trials continue. New strategies targeting the protein at the level of transcription, translation, and posttranslational modification and aggregation engender new hope that a successful strategy will emerge, but there is much work ahead. Some of the clinical manifestations of the illness, particularly chorea, affective symptoms, and irritability, are amenable to palliative strategies, but physicians have a poor evidence base on which to select the best agents. Clinical trials since 2013 have dashed hopes that coenzyme Q10 or creatine might have disease-modifying properties but suggested other agents were safe or hinted at efficacy (cysteamine, selisistat, hydroxyquinoline) and could proceed into later-stage disease modification trials. The hunt for effective symptom relief suggested that pridopidine might be shown effective given the right outcome measure. This review summarizes recent progress in HD and highlights promising new strategies for slowing disease progression and relieving suffering in HD. © 2015 International Parkinson and Movement Disorder Society.

  18. Huntington's disease: a clinical review.

    Science.gov (United States)

    McColgan, Peter; Tabrizi, Sarah J

    2017-08-17

    Huntington's disease (HD) is a fully penetrant neurodegenerative disease caused by a dominantly inherited CAG trinucleotide repeat expansion in the huntingtin gene on chromosome 4. In Western populations HD has a prevalence of 10.6-13.7 individuals per 100,000. It is characterised by cognitive, motor and psychiatric disturbance. At the cellular level mutant huntingtin results in neuronal dysfunction and death through a number of mechanisms, including disruption of proteostasis, transcription and mitochondrial function and direct toxicity of the mutant protein. Early macroscopic changes are seen in the striatum with involvement of the cortex as the disease progresses. There are currently no disease modifying treatments therefore supportive and symptomatic management is the mainstay of treatment. In recent years there have been significant advances in understanding both the cellular pathology and the macroscopic structural brain changes that occur as the disease progresses. In the last decade there has been a large growth in potential therapeutic targets and clinical trials. Perhaps the most promising of these are the emerging therapies aimed at lowering levels of mutant huntingtin. Antisense oligonucleotide therapy is one such approach with clinical trials currently underway. This may bring us one step closer to treating and potentially preventing this devastating condition. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

  19. Movement sequencing in Huntington disease.

    Science.gov (United States)

    Georgiou-Karistianis, Nellie; Long, Jeffrey D; Lourens, Spencer G; Stout, Julie C; Mills, James A; Paulsen, Jane S

    2014-08-01

    To examine longitudinal changes in movement sequencing in prodromal Huntington's disease (HD) participants (795 prodromal HD; 225 controls) from the PREDICT-HD study. Prodromal HD participants were tested over seven annual visits and were stratified into three groups (low, medium, high) based on their CAG-Age Product (CAP) score, which indicates likely increasing proximity to diagnosis. A cued movement sequence task assessed the impact of advance cueing on response initiation and execution via three levels of advance information. Compared to controls, all CAP groups showed longer initiation and movement times across all conditions at baseline, demonstrating a disease gradient for the majority of outcomes. Across all conditions, the high CAP group had the highest mean for baseline testing, but also demonstrated an increase in movement time across the study. For initiation time, the high CAP group showed the highest mean baseline time across all conditions, but also faster decreasing rates of change over time. With progress to diagnosis, participants may increasingly use compensatory strategies, as evidenced by faster initiation. However, this occurred in conjunction with slowed execution times, suggesting a decline in effectively accessing control processes required to translate movement into effective execution.

  20. Language impairment in Huntington's disease

    Directory of Open Access Journals (Sweden)

    Mariana Jardim Azambuja

    2012-06-01

    Full Text Available Language alterations in Huntington's disease (HD are reported, but their nature and correlation with other cognitive impairments are still under investigation. This study aimed to characterize the language disturbances in HD and to correlate them to motor and cognitive aspects of the disease. We studied 23 HD patients and 23 controls, matched for age and schooling, using the Boston Diagnostic Aphasia Examination, Boston Naming Test, the Token Test, Animal fluency, Action fluency, FAS-COWA, the Symbol Digit Modalities Test, the Stroop Test and the Hooper Visual Organization Test (HVOT. HD patients performed poorer in verbal fluency (p<0.0001, oral comprehension (p<0.0001, repetition (p<0.0001, oral agility (p<0.0001, reading comprehension (p=0.034 and narrative writing (p<0.0001. There was a moderate correlation between the Expressive Component and Language Competency Indexes and the HVOT (r=0.519, p=0.011 and r=0.450, p=0.031, respectively. Language alterations in HD seem to reflect a derangement in both frontostriatal and frontotemporal regions.

  1. Unusual early-onset Huntingtons disease.

    Science.gov (United States)

    Vargas, Antonio P; Carod-Artal, Francisco J; Bomfim, Denise; Vázquez-Cabrera, Carolina; Dantas-Barbosa, Carmela

    2003-06-01

    Huntington's disease is an autosomal dominant progressive neurodegenerative disorder characterized by involuntary movements, cognitive decline, and behavioral disorders leading to functional disability. In contrast to patients with adult onset, in which chorea is the major motor abnormality, children often present with spasticity, rigidity, and significant intellectual decline associated with a more rapidly progressive course. An unusual early-onset Huntington's disease case of an 11-year-old boy with severe hypokinetic/rigid syndrome appearing at the age of 2.5 years is presented. Clinical diagnosis was confirmed by polymerase chain reaction study of the expanded IT-15 allele with a compatible size of 102 cytosine-adenosine-guanosine repeats L-Dopa mildly ameliorated rigidity, bradykinesia, and dystonia. We conclude that Huntington's disease should be included in the differential diagnoses of regressive syndromes of early childhood.

  2. Development of biomarkers for Huntington's disease.

    Science.gov (United States)

    Weir, David W; Sturrock, Aaron; Leavitt, Blair R

    2011-06-01

    Huntington's disease is an autosomal dominant, progressive neurodegenerative disorder, for which there is no disease-modifying treatment. By use of predictive genetic testing, it is possible to identify individuals who carry the gene defect before the onset of symptoms, providing a window of opportunity for intervention aimed at preventing or delaying disease onset. However, without robust and practical measures of disease progression (ie, biomarkers), the efficacy of therapeutic interventions in this premanifest Huntington's disease population cannot be readily assessed. Current progress in the development of biomarkers might enable evaluation of disease progression in individuals at the premanifest stage of the disease; these biomarkers could be useful in defining endpoints in clinical trials in this population. Clinical, cognitive, neuroimaging, and biochemical biomarkers are being investigated for their potential in clinical use and their value in the development of future treatments for patients with Huntington's disease.

  3. Monkey hybrid stem cells develop cellular features of Huntington's disease

    Directory of Open Access Journals (Sweden)

    Lorthongpanich Chanchao

    2010-02-01

    Full Text Available Abstract Background Pluripotent stem cells that are capable of differentiating into different cell types and develop robust hallmark cellular features are useful tools for clarifying the impact of developmental events on neurodegenerative diseases such as Huntington's disease. Additionally, a Huntington's cell model that develops robust pathological features of Huntington's disease would be valuable for drug discovery research. Results To test this hypothesis, a pluripotent Huntington's disease monkey hybrid cell line (TrES1 was established from a tetraploid Huntington's disease monkey blastocyst generated by the fusion of transgenic Huntington's monkey skin fibroblast and a wild-type non-transgenic monkey oocyte. The TrES1 developed key Huntington's disease cellular pathological features that paralleled neural development. It expressed mutant huntingtin and stem cell markers, was capable of differentiating to neural cells, and developed teratoma in severely compromised immune deficient (SCID mice. Interestingly, the expression of mutant htt, the accumulation of oligomeric mutant htt and the formation of intranuclear inclusions paralleled neural development in vitro , and even mutant htt was ubiquitously expressed. This suggests the development of Huntington's disease cellular features is influenced by neural developmental events. Conclusions Huntington's disease cellular features is influenced by neural developmental events. These results are the first to demonstrate that a pluripotent stem cell line is able to mimic Huntington's disease progression that parallels neural development, which could be a useful cell model for investigating the developmental impact on Huntington's disease pathogenesis.

  4. Maternal transmission in sporadic Huntington's disease.

    OpenAIRE

    Sánchez, A; Milà, M.; Castellví-Bel, S; Rosich, M; Jiménez, D; Badenas, C.; ESTIVILL, X.

    1997-01-01

    Huntington's disease is an autosomal dominant neurodegenerative disorder caused by the expansion of a (CAG)n repeat in the IT15 gene. Three per cent of cases are sporadic and in those in which family studies have been performed, the origin of the mutation was always paternal. The first sporadic case of Huntington's disease is presented in which a premutated maternal allele of 37 CAG repeats was transmitted expanded to the proband (43 CAG repeats). Molecular analysis of the IT15 gene is extrem...

  5. Paleoecology of the Springfield Coal Member (Desmoinesian, Illinois Basin) near the Leslie Cemetery paleochannel, southwestern Indiana

    Energy Technology Data Exchange (ETDEWEB)

    Willard, D.B.; DiMichele, W.A.; Eggert, D.L.; Hower, J.C.; Rexroad, C.B.; Scott, A.C. [US Geological Survey, Reston, VA (United States)

    1995-01-01

    The Springfield Coal Member (Carbondale Group, Petersburg Formation of Indiana) is split locally in Warrick and Gibson Counties, Indiana, by clastic rocks of the Folsomville Member (Carbondale Group, Petersburg Formation) that represent the Leslie Cemetery paleochannel, part of a large, interconnected paleochannel system in the Springfield coal bed. This study incorporated analysis of miospore and megaspore assemblages, coal petrography, plant compression fossils and conodonts from the coal and clastic split to document changes in the swamp and its vegetation in response to the activity of the Leslie Cemetery paleochannel. Palynological and petrographic data indicate that environmental conditions and vegetation in the lower bench of coal near the Leslie Cemetery paleochannel were similar to those found in profiles through the coal bed at sites near the larger, more extensive Galatia paleochannel. Higher than normal salinity levels may explain the vegetational changes observed in the upper bench of coal near the Leslie Cemetery paleochannel. 76 refs., 11 figs., 9 tabs.

  6. OrthoImagery submittal for Greene County, Indiana

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — Digital orthographic imagery datasets contain georeferenced images of the Earth?s surface, collected by a sensor in which object displacement has been removed for...

  7. OrthoImagery submittal for Posey County, Indiana

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — Digital orthographic imagery datasets contain georeferenced images of the Earth?s surface, collected by a sensor in which object displacement has been removed for...

  8. OrthoImagery submittal for Blackford County, Indiana

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — Digital orthographic imagery datasets contain georeferenced images of the Earth's surface, collected by a sensor in which object displacement has been removed for...

  9. OrthoImagery submittal for Rush County, Indiana

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — Digital orthographic imagery datasets contain georeferenced images of the Earth's surface, collected by a sensor in which object displacement has been removed for...

  10. OrthoImagery submittal for Switzerland County, Indiana

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — Digital orthographic imagery datasets contain georeferenced images of the Earth's surface, collected by a sensor in which object displacement has been removed for...

  11. OrthoImagery submittal for Hamilton County, Indiana

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — Digital orthographic imagery datasets contain georeferenced images of the Earth?s surface, collected by a sensor in which object displacement has been removed for...

  12. OrthoImagery submittal for Wells County, Indiana

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — Digital orthographic imagery datasets contain georeferenced images of the Earth's surface, collected by a sensor in which object displacement has been removed for...

  13. OrthoImagery submittal for Cass County, Indiana

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — Digital orthographic imagery datasets contain georeferenced images of the Earth's surface, collected by a sensor in which object displacement has been removed for...

  14. OrthoImagery submittal for Henry County, Indiana

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — Digital orthographic imagery datasets contain georeferenced images of the Earth?s surface, collected by a sensor in which object displacement has been removed for...

  15. DIGITAL FLOOD INSURANCE RATE MAP DATABASE, HAMILTON COUNTY, INDIANA, USA

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — The Digital Flood Insurance Rate Map (DFIRM) Database depicts flood risk Information And supporting data used to develop the risk data. The primary risk;...

  16. OrthoImagery submittal for Lagrange County, Indiana

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — Digital orthographic imagery datasets contain georeferenced images of the Earth's surface, collected by a sensor in which object displacement has been removed for...

  17. DIGITAL FLOOD INSURANCE RATE MAP DATABASE, CLARK COUNTY, INDIANA, USA

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — The Digital Flood Insurance Rate Map (DFIRM) Database depicts flood risk Information And supporting data used to develop the risk data. The primary risk;...

  18. OrthoImagery submittal for Fulton County, Indiana

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — Digital orthographic imagery datasets contain georeferenced images of the Earth's surface, collected by a sensor in which object displacement has been removed for...

  19. OrthoImagery submittal for Orange County, Indiana

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — Digital orthographic imagery datasets contain georeferenced images of the Earth's surface, collected by a sensor in which object displacement has been removed for...

  20. OrthoImagery submittal for Carroll County, Indiana

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — Digital orthographic imagery datasets contain georeferenced images of the Earth's surface, collected by a sensor in which object displacement has been removed for...

  1. DIGITAL FLOOD INSURANCE RATE MAP DATABASE, ALLEN COUNTY, INDIANA, USA

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — The Digital Flood Insurance Rate Map (DFIRM) Database depicts flood risk Information And supporting data used to develop the risk data. The primary risk;...

  2. DIGITAL FLOOD INSURANCE RATE MAP DATABASE, BOONE COUNTY, INDIANA, USA

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — The Digital Flood Insurance Rate Map (DFIRM) Database depicts flood risk Information And supporting data used to develop the risk data. The primary risk;...

  3. OrthoImagery submittal for White County, Indiana

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — Digital orthographic imagery datasets contain georeferenced images of the Earth's surface, collected by a sensor in which object displacement has been removed for...

  4. Landfills, Hazardous Waste - WASTE_INDUSTRIAL_IDEM_IN: Industrial Waste Sites in Indiana (Indiana Department of Environmental Management, Point Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — WASTE_INDUSTRIAL_IDEM_IN is a point shapefile that contains industrial waste site locations in Indiana, provided by personnel of Indiana Department of Environmental...

  5. Airports and Airfields - AIRPORTS_PUBLIC_INDOT_IN: Public-Use Airports in Indiana (Indiana Department of Transportation, Point Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — AIRPORTS_PUBLIC_INDOT_IN is a point shapefile that shows public-use airports in Indiana. It is derived from data provided by personnel of the Indiana Department of...

  6. Land Use and Land Cover - BROWNFIELDS_IDEM_IN: Brownfield Locations in Indiana (Indiana Department of Environmental Management, Point Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — BROWNFIELDS_IDEM_IN is a point shapefile that contains brownfield locations in Indiana, provided by personnel of Indiana Department of Environmental Management,...

  7. Railroad Lines - RAIL_CROSSINGS_INDOT_IN: Railroad Crossings in Indiana (Indiana Department of Transportation, 1:1200, Point Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — RAIL_CROSSINGS_INDOT_IN is a point shapefile that contains locations of rail crossings in Indiana, provided by personnel of Indiana Department of Transportation...

  8. Road and Street Centerlines - RAMPS_INDOT_IN: Ramp System in Indiana (Indiana Department of Transportation, Line Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — RAMPS_INDOT_IN is a line shapefile that contains all ramps in Indiana, provided by personnel of Indiana Department of Transportation (INDOT), Business Information...

  9. Landfills - OPEN_DUMPS_IDEM_IN: Open Dump Sites in Indiana (Indiana Department of Environmental Management, Point Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — OPEN_DUMPS_IDEM_IN is a point shapefile that contains open dump site locations in Indiana, provided by personnel of Indiana Department of Environmental Management,...

  10. 2008 Public Opinion Survey on K-12 Education in Indiana

    Science.gov (United States)

    Plucker, Jonathan A.; Spradlin, Terry E.; Burroughs, Nathan A.; Hiller, Stephen C.

    2008-01-01

    During the course of each calendar year since 2003, staff of the Center for Evaluation & Education Policy (CEEP) at Indiana University evaluates the benefits of continuing the Annual Public Opinion Survey on K-12 Education in Indiana. In 2008, the Indiana legislature determined that school corporations would no longer use property tax revenues…

  11. Pathogenic insights from Huntington's disease-like 2 and other Huntington's disease genocopies.

    Science.gov (United States)

    Margolis, Russell L; Rudnicki, Dobrila D

    2016-12-01

    Huntington's disease-like 2 (HDL2) is a rare, progressive, autosomal dominant neurodegenerative disorder that genetically, clinically, and pathologically closely resembles Huntington's disease. We review HDL2 pathogenic mechanisms and examine the implications of these mechanisms for Huntington's disease and related diseases. HDL2 is caused by a CTG/CAG repeat expansion in junctophilin-3. Available data from cell and animal models and human brain suggest that HDL2 is a complex disease in which transcripts and proteins expressed bidirectionally from the junctophilin-3 locus contribute to pathogenesis through both gain-and loss-of-function mechanisms. Recent advances indicate that the pathogenesis of Huntington's disease is equally complex, despite the emphasis on toxic gain-of-function properties of the mutant huntingtin protein. Studies examining in parallel the genetic, clinical, neuropathological, and mechanistic similarities between Huntington's disease and HDL2 have begun to identify points of convergence between the pathogenic pathways of the two diseases. Comparisons to other diseases that are phenotypically or genetically related to Huntington's disease and HDL2 will likely reveal additional common pathways. The ultimate goal is to identify shared therapeutic targets and eventually develop therapies that may, at least in part, be effective across multiple similar rare diseases, an essential approach given the scarcity of resources for basic and translational research.

  12. Hydrology of area 32, Eastern Region, Interior Coal Province, Indiana

    Science.gov (United States)

    Wangsness, David J.; Miller, R.L.; Bailey, Z.C.; Crawford, Charles G.

    1981-01-01

    Hydrologic and water-quality information from the coal region in parts of 11 counties in southwestern Indiana are summarized. Pennsylvanian and Mississippian bedrock are overlain by drift and till from the Kansan, Illinoian, and Wisconsinan glaciers that covered two-thirds of the area. Most of the coal is mined from Pennsylvanian coal units. As of 1978, more than 1-billion short tons of coal had been mined from the 11 counties. More than 12-billion short tons remained in reserve. During 1978, 17.8-million short tons were mined. Coal mining disturbs less than 1% of the land in the study area. Sixty-two percent of the land use is agricultural, and 29% is forested. More coal is mined in the southern and western parts of the study area than elsewhere. Surface water in these parts had lower pH and alkalinity than water in the north and east parts. Specific conductance and concentrations of sulfate, iron, and manganese in surface water were higher in the south and west than elsewhere. (USGS)

  13. Impaired motor speech performance in Huntington's disease.

    Science.gov (United States)

    Skodda, Sabine; Schlegel, Uwe; Hoffmann, Rainer; Saft, Carsten

    2014-04-01

    Dysarthria is a common symptom of Huntington's disease and has been reported, besides other features, to be characterized by alterations of speech rate and regularity. However, data on the specific pattern of motor speech impairment and their relationship to other motor and neuropsychological symptoms are sparse. Therefore, the aim of the present study was to describe and objectively analyse different speech parameters with special emphasis on the aspect of speech timing of connected speech and non-speech verbal utterances. 21 patients with manifest Huntington's disease and 21 age- and gender-matched healthy controls had to perform a reading task and several syllable repetition tasks. Computerized acoustic analysis of different variables for the measurement of speech rate and regularity generated a typical pattern of impaired motor speech performance with a reduction of speech rate, an increase of pauses and a marked disability to steadily repeat single syllables. Abnormalities of speech parameters were more pronounced in the subgroup of patients with Huntington's disease receiving antidopaminergic medication, but were also present in the drug-naïve patients. Speech rate related to connected speech and parameters of syllable repetition showed correlations to overall motor impairment, capacity of tapping in a quantitative motor assessment and some score of cognitive function. After these preliminary data, further investigations on patients in different stages of disease are warranted to survey if the analysis of speech and non-speech verbal utterances might be a helpful additional tool for the monitoring of functional disability in Huntington's disease.

  14. Destination and source memory in Huntington's disease

    NARCIS (Netherlands)

    El Haj, M.; Caillaud, M.; Verny, C.; Fasotti, L.; Allain, P.

    2016-01-01

    Destination memory refers to the recall of the destination of previously relayed information, and source memory refers to the recollection of the origin of received information. We compared both memory systems in Huntington's disease (HD) participants. For this, HD participants and healthy adults

  15. Kas Huntington oli prohvet? / Priit Simson

    Index Scriptorium Estoniae

    Simson Priit, 1977-

    2008-01-01

    Autor käsitleb Samuel Huntingtoni teese ning leiab, et tegelikult Huntington ei pakkunud õigustust islamiriikide ründamisele, vaid pigem hoiatas tsivilisatsioonide siseasjusse sekkumise, tekkida võiva ahelreaktsiooni eest, kus üks tsivilisatsiooni liige tõmbab sõtta ka teise

  16. Wearable Sensors in Huntington Disease: A Pilot Study.

    Science.gov (United States)

    Andrzejewski, Kelly L; Dowling, Ariel V; Stamler, David; Felong, Timothy J; Harris, Denzil A; Wong, Cynthia; Cai, Hang; Reilmann, Ralf; Little, Max A; Gwin, Joseph T; Biglan, Kevin M; Dorsey, E Ray

    2016-06-18

    The Unified Huntington's Disease Rating Scale (UHDRS) is the principal means of assessing motor impairment in Huntington disease but is subjective and generally limited to in-clinic assessments. To evaluate the feasibility and ability of wearable sensors to measure motor impairment in individuals with Huntington disease in the clinic and at home. Participants with Huntington disease and controls were asked to wear five accelerometer-based sensors attached to the chest and each limb for standardized, in-clinic assessments and for one day at home. A second chest sensor was worn for six additional days at home. Gait measures were compared between controls, participants with Huntington disease, and participants with Huntington disease grouped by UHDRS total motor score using Cohen's d values. Fifteen individuals with Huntington disease and five controls completed the study. Sensor data were successfully captured from 18 of the 20 participants at home. In the clinic, the standard deviation of step time (time between consecutive steps) was increased in Huntington disease (p Huntington disease, and participants with Huntington disease grouped by motor impairment.

  17. Silencing Huntington's chorea: Is RNA Interference a Potential Cure?

    Directory of Open Access Journals (Sweden)

    Gerlinde A. Metz

    2006-01-01

    Full Text Available In 1872, George Huntington described Huntington's disease as characterized by motor, cognitive and psychiatric impairments. Huntington's disease is a dominant and autosomal mutation on chromosome 4 featuring the insertion of numerous CAG repeats. CAG codes for the amino acid, glutmanine that forms part of the Huntingtin protein (htt. Excess glutamine attachments make htt prone to accumulate in neurons. Three genes can be considered when developing therapies for Huntington's disease. They include targeting the symptoms of the disease, the progression of the disease and the cause of the disease. By using RNA interference (RNAi, the cause of the disease can be targeted. RNAi is a method that could potentially silence the formation of abnormal htt. This paper will discuss how RNAi could potentially cure Huntington's disease, by describing the genetic and proteinomic basis of Huntington's disease, the function of RNAi in Huntington's disease and the problems of benefits of RNAi. Preliminary work using RNAi in transgenic mice has shown a decrease in the behavioural expression of the mutant Huntington gene. There are several limitations associated with using RNAi as a gene therapy. For example, the effects of RNAi are short lived. A transposition system such as Sleeping Beauty can be used to increase the integration of the gene, however, for patients who currently have Huntington's disease, RNAi may potentially be used in combination with drugs or other treatments to target both symptoms and the underlying cause of Huntington's disease. This combination could eventually alleviate many painful symptoms associated with Huntington's disease and could even stop the progressive neurodegeneration of Huntington's disease. This review concludes that a substantial amount of new research is still necessary before RNAi is directly applicable to human patients with Huntington's disease.

  18. Parcels and Land Ownership - MANAGED_LANDS_IDNR_IN: Managed Lands in Indiana (Indiana Department of Natural Resources, 1:24,000, Polygon Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — Managed_Lands_IDNR_IN is a polygon shapefile that contains managed land areas in Indiana, provided by personnel of the Indiana Natural Heritage Data Center, Indiana...

  19. Water resources data, Indiana, water year 1993

    Science.gov (United States)

    Stewart, James A.; Keeton, Charles R.; Benedict, Brian L.; Hammil, Lowell E.

    1994-01-01

    Water resources data for the 1993 water year for Indiana consist of records of discharge, stage, and water quality of streams and wells; reservoir stage and contents; and water levels in lakes and wells. This report contains records of discharge for 175 stream-gaging station, stage for 5 stream station, 1 sediment station, stage and contents for 1 reservoir, water quality for 3 streams, and water levels for 80 lakes and 94 observation wells. These data represent that part of the National Water Data System operated by the U.S. Geological Survey in Indiana in cooperation with State and Federal agencies.

  20. Water resources data, Indiana, water year 1992

    Science.gov (United States)

    Stewart, James A.; Keeton, Charles R.; Benedict, Brian L.; Hammil, Lowell E.

    1993-01-01

    Water resources data for the 1992 water year for Indiana consist of records of discharge, stage, and water quality of streams and wells; reservoir stage and contents; and water levels in lakes and wells. This report contains records of discharge for 175 stream-gaging stations, stage for 7 stream stations, 1 sediment station, stage and contents for 1 reservoir, water quality for 3 streams, and water levels for 80 lakes and 94 observation wells. These data represent that part of the National Water Data System operated by the U.S. Geological Survey in Indiana in cooperation with State and Federal agencies.

  1. Water resources data, Indiana, water year 1991

    Science.gov (United States)

    Stewart, James A.; Deiwert, Clyde E.

    1992-01-01

    Water resources data for the 1991 water year for Indiana consist of records of discharge, stage, and water quality of streams and wells; reservoir stage and contents; and water levels in lakes and wells. This report contains records of discharge for 183 stream-gaging stations, stage for 7 stream stations, stage and contents for 1 reservoir, water quality for 3 streams, and water levels for 80 lakes and 95 observation wells. These data represent that part of the National Water Data System operated by the U.S. Geological Survey in Indiana in cooperation with State and Federal Agencies.

  2. A Metabolic Study of Huntington's Disease.

    Directory of Open Access Journals (Sweden)

    Rajasree Nambron

    Full Text Available Huntington's disease patients have a number of peripheral manifestations suggestive of metabolic and endocrine abnormalities. We, therefore, investigated a number of metabolic factors in a 24-hour study of Huntington's disease gene carriers (premanifest and moderate stage II/III and controls.Control (n = 15, premanifest (n = 14 and stage II/III (n = 13 participants were studied with blood sampling over a 24-hour period. A battery of clinical tests including neurological rating and function scales were performed. Visceral and subcutaneous adipose distribution was measured using magnetic resonance imaging. We quantified fasting baseline concentrations of glucose, insulin, cholesterol, triglycerides, lipoprotein (a, fatty acids, amino acids, lactate and osteokines. Leptin and ghrelin were quantified in fasting samples and after a standardised meal. We assessed glucose, insulin, growth hormone and cortisol concentrations during a prolonged oral glucose tolerance test.We found no highly significant differences in carbohydrate, protein or lipid metabolism markers between healthy controls, premanifest and stage II/III Huntington's disease subjects. For some markers (osteoprotegerin, tyrosine, lysine, phenylalanine and arginine there is a suggestion (p values between 0.02 and 0.05 that levels are higher in patients with premanifest HD, but not moderate HD. However, given the large number of statistical tests performed interpretation of these findings must be cautious.Contrary to previous studies that showed altered levels of metabolic markers in patients with Huntington's disease, our study did not demonstrate convincing evidence of abnormalities in any of the markers examined. Our analyses were restricted to Huntington's disease patients not taking neuroleptics, anti-depressants or other medication affecting metabolic pathways. Even with the modest sample sizes studied, the lack of highly significant results, despite many being tested, suggests that

  3. County Spending

    Data.gov (United States)

    Montgomery County of Maryland — This dataset includes County spending data for Montgomery County government. It does not include agency spending. Data considered sensitive or confidential and will...

  4. 40 CFR 81.64 - Huntington (West Virginia)-Ashland (Kentucky)-Portsmouth-Ironton (Ohio) Interstate Air Quality...

    Science.gov (United States)

    2010-07-01

    ... County, Elliott County, Fleming County, Greenup County, Lawrence County, Lewis County, Mason County, Menifee County, Montgomery County, Morgan County, Robertson County, Rowan County. In the State of...

  5. Cultural Resources - MUSEUMS_IGS_IN: Museums in Indiana (Indiana Geological Survey, Point Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — MUSEUMS_IGS_IN is a point shapefile showing the locations of 118 museums in Indiana. Using street addresses obtained from Web pages, a shapefile was created using...

  6. Geophysical Features - STRUCTURAL_FEATURES_IN: Structural Features of Indiana (Indiana Geological Survey, Line Shapefile)

    Data.gov (United States)

    NSGIC State | GIS Inventory — STRUCTURAL_FEATURES_IN is a line shapefile that shows the location of known structural features in Indiana; source data scales range from 1:12,000 to 1:500,000 (only...

  7. A survey of bees (hymenoptera: Apoidea) of the Indiana dunes and Northwest Indiana, USA

    Science.gov (United States)

    Grundel, R.; Jean, R.P.; Frohnapple, K.J.; Gibbs, J.; Glowacki, G.A.; Pavlovic, N.B.

    2011-01-01

    The Indiana Dunes, and nearby natural areas in northwest Indiana, are floristically rich Midwest U.S. locales with many habitat types. We surveyed bees along a habitat gradient ranging from grasslands to forests in these locales, collecting at least 175 bee species along this gradient plus 29 additional species in other nearby habitats. About 25% of all species were from the genus Lasioglossum and 12% of the species were associated with sandy soils. Several bumblebee (Bombus) species of conservation concern that should occur in this region were not collected during our surveys. Similarity of the northwest Indiana bee fauna to other published U.S. faunas decreased about 1.3% per 100 km distance from northwest Indiana. Thirty percent of bees netted from flowers were males. Males and females differed significantly in their frequency of occurrence on different plant species. For bees collected in bowl traps, the percentage captured in fluorescent yellow traps declined and in fluorescent blue traps increased from spring to late summer. Capture rates for different bee genera varied temporally, with about a quarter of the genera being captured most frequently in late spring and a quarter in late summer. Capture rates for most genera were higher in more open than in more closed canopy habitats. The maximum number of plant species on which a single bee species was captured plateaued at 24, on average. Forty-nine percent of bee species known to occur in Indiana were found at these northwest Indiana sites. Having this relatively high proportion of the total Indiana bee fauna is consistent with Indiana Dunes existing at a biogeographic crossroads where grassland and forest biomes meet in a landscape whose climate and soils are affected by proximity to Lake Michigan. The resulting habitat, plant, edaphic, and climatic diversity likely produces the diverse bee community documented.

  8. Molecular diagnostic analysis for Huntington's disease: a prospective evaluation.

    OpenAIRE

    MacMillan, J C; Davies, P.; Harper, P S

    1995-01-01

    The availability of mutation analysis for the CAG repeat expansion associated with Huntington's disease has prompted clinicians in various specialties to request testing of samples from patients displaying clinical features that might be attributable to Huntington's disease. A series of 38 cases presenting with clinical features thought possibly to be due to Huntington's disease were analysed prospectively. In 53% of such cases presenting initially with chorea and 62.5% with psychiatric sympt...

  9. Rural Indiana Profile: Alcohol, Tobacco & Other Drugs.

    Science.gov (United States)

    Drug Strategies, Washington, DC.

    This report examines alcohol, tobacco, and other drug use in rural parts of Indiana, as well as public and private initiatives to reduce these problems. The report is based on epidemiological, health, and criminal justice indicators; focus groups; and in-depth interviews with local officials, researchers, service providers, and civic leaders.…

  10. 77 FR 51101 - Indiana Disaster #IN-00048

    Science.gov (United States)

    2012-08-23

    ... From the Federal Register Online via the Government Publishing Office ] SMALL BUSINESS ADMINISTRATION Indiana Disaster IN-00048 AGENCY: U.S. Small Business Administration. ACTION: Notice. SUMMARY... INFORMATION CONTACT: A. Escobar, Office of Disaster Assistance, U.S. Small Business Administration, 409...

  11. 76 FR 77579 - Indiana Disaster #IN-00039

    Science.gov (United States)

    2011-12-13

    ... ADMINISTRATION Indiana Disaster IN-00039 AGENCY: U.S. Small Business Administration. ACTION: Notice. SUMMARY...: 09/07/2012. ADDRESSES: Submit completed loan applications to: U.S. Small Business Administration... CONTACT: A. Escobar, Office of Disaster Assistance, U.S. Small Business Administration, 409 3rd Street...

  12. State Teacher Policy Yearbook, 2009. Indiana

    Science.gov (United States)

    National Council on Teacher Quality, 2009

    2009-01-01

    This Indiana's edition of the National Council on Teacher Quality's (NCTQ's) 2009 "State Teacher Policy Yearbook" is the third annual look at state policies impacting the teaching profession. It is hoped that this report will help focus attention on areas where state policymakers can make changes that will have a positive impact on…

  13. Normal CAG and CCG repeats in the Huntington`s disease genes of Parkinson`s disease patients

    Energy Technology Data Exchange (ETDEWEB)

    Rubinsztein, D.C.; Leggo, J.; Barton, D.E. [Cambridge Univ. (United Kingdom)] [and others

    1995-04-24

    The clinical features of Parkinson`s disease, particularly rigidity and bradykinesia and occasionally tremor, are seen in juvenile-onset Huntington`s disease. Therefore, the CAG and CCG repeats in the Huntington`s disease gene were investigated in 45 Parkinson`s disease patients and compared to 40 control individuals. All of the Parkinson`s disease chromosomes fell within the normal size ranges. In addition, the distributions of the two repeats in the Parkinson`s disease patients did not differ significantly from those of the control population. Therefore, abnormalities of these trinucleotide repeats in the Huntington`s disease gene are not likely to contribute to the pathogenesis of Parkinson`s disease. 12 refs., 2 figs.

  14. Indiana Studies: Hoosier History, Government, and People. Unit III: From Sectional Division to Political Unity.

    Science.gov (United States)

    Barger, Harry D.; And Others

    Unit 3 of a six-unit series on Indiana state history designed to be taught in Indiana secondary schools tells the story of Indiana from 1829 to 1908. Chapter 1 discusses national issues in an Indiana context. The effects of social movements such as Abolition, the underground railroad, and the Fugitive Slave Law on Indiana politics are examined.…

  15. Large genetic animal models of Huntington's Disease.

    Science.gov (United States)

    Morton, A Jennifer; Howland, David S

    2013-01-01

    The dominant nature of the Huntington's disease gene mutation has allowed genetic models to be developed in multiple species, with the mutation causing an abnormal neurological phenotype in all animals in which it is expressed. Many different rodent models have been generated. The most widely used of these, the transgenic R6/2 mouse, carries the mutation in a fragment of the human huntingtin gene and has a rapidly progressive and fatal neurological phenotype with many relevant pathological changes. Nevertheless, their rapid decline has been frequently questioned in the context of a disease that takes years to manifest in humans, and strenuous efforts have been made to make rodent models that are genetically more 'relevant' to the human condition, including full length huntingtin gene transgenic and knock-in mice. While there is no doubt that we have learned, and continue to learn much from rodent models, their usefulness is limited by two species constraints. First, the brains of rodents differ significantly from humans in both their small size and their neuroanatomical organization. Second, rodents have much shorter lifespans than humans. Here, we review new approaches taken to these challenges in the development of models of Huntington's disease in large brained, long-lived animals. We discuss the need for such models, and how they might be used to fill specific niches in preclinical Huntington's disease research, particularly in testing gene-based therapeutics. We discuss the advantages and disadvantages of animals in which the prodromal period of disease extends over a long time span. We suggest that there is considerable 'value added' for large animal models in preclinical Huntington's disease research.

  16. Revisiting the neuropsychiatry of Huntington's disease

    Directory of Open Access Journals (Sweden)

    Antonio Lucio Teixeira

    Full Text Available ABSTRACT Huntington's disease (HD is an autosomal dominant neurodegenerative disease classified under the choreas. Besides motor symptoms, HD is marked by cognitive and behavioral symptoms, impacting patients' functional capacity. The progression of cognitive impairment and neuropsychiatric symptoms occur in parallel with neurodegeneration. The nature of these symptoms is very dynamic, and the major clinical challenges include executive dysfunction, apathy, depression and irritability. Herein, we provide a focused updated review on the cognitive and psychiatric features of HD.

  17. [Sporadic juvenile forms of Huntington's chorea].

    Science.gov (United States)

    Zinchenko, A P; Goncharov, V D; Burtianskii, D L; Zakhar'ev, Iu M

    1980-01-01

    Six patients with Huntington's chorea in the age of 15-24 years old, suffered from diffusive choreic hyperkynesis with slowly progressive dementia. The development of this disease in childhood and adolescence was atypical, as nobody in the family and in kin sufferred from it and it was difficult to diagnose the disease. Recognition of the disease was promoted by pneumoencephalography, electromyography and memory investigation.

  18. 77 FR 41954 - Approval and Promulgation of Air Quality Implementation Plans; Indiana

    Science.gov (United States)

    2012-07-17

    ... submitted by the Indiana Department of Environmental Management on May 14, 2012, to revise the Indiana State Implementation Plan (SIP). The submission revises the Indiana Administrative Code (IAC) definition of...

  19. Väike psühhoanalüüs Indiana Jonesile / Aarne Ruben

    Index Scriptorium Estoniae

    Ruben, Aarne, 1971-

    2008-01-01

    Steven Spielbergi neljas Indiana Jones'i film Harrison Fordiga nimiosas "Indiana Jones ja kristallpealuu kuningriik" ("Indiana Jones and the Kingdom of the Crystal Skull") : Ameerika Ühendriigid 2008

  20. 78 FR 14791 - Public Water System Supervision Program Approval for the State of Indiana

    Science.gov (United States)

    2013-03-07

    ... AGENCY Public Water System Supervision Program Approval for the State of Indiana AGENCY: Environmental... has tentatively approved three revisions to the State of Indiana's public water system supervision... the State of Indiana's public water system supervision program, thereby giving IDEM...

  1. Väike psühhoanalüüs Indiana Jonesile / Aarne Ruben

    Index Scriptorium Estoniae

    Ruben, Aarne, 1971-

    2008-01-01

    Steven Spielbergi neljas Indiana Jones'i film Harrison Fordiga nimiosas "Indiana Jones ja kristallpealuu kuningriik" ("Indiana Jones and the Kingdom of the Crystal Skull") : Ameerika Ühendriigid 2008

  2. Altered cholesterol and fatty acid metabolism in Huntington disease.

    Science.gov (United States)

    Block, Robert C; Dorsey, E Ray; Beck, Christopher A; Brenna, J Thomas; Shoulson, Ira

    2010-01-01

    Huntington disease is an autosomal dominant neurodegenerative disorder characterized by behavioral abnormalities, cognitive decline, and involuntary movements that lead to a progressive decline in functional capacity, independence, and ultimately death. The pathophysiology of Huntington disease is linked to an expanded trinucleotide repeat of cytosine-adenine-guanine (CAG) in the IT-15 gene on chromosome 4. There is no disease-modifying treatment for Huntington disease, and novel pathophysiological insights and therapeutic strategies are needed. Lipids are vital to the health of the central nervous system, and research in animals and humans has revealed that cholesterol metabolism is disrupted in Huntington disease. This lipid dysregulation has been linked to specific actions of the mutant huntingtin on sterol regulatory element binding proteins. This results in lower cholesterol levels in affected areas of the brain with evidence that this depletion is pathologic. Huntington disease is also associated with a pattern of insulin resistance characterized by a catabolic state resulting in weight loss and a lower body mass index than individuals without Huntington disease. Insulin resistance appears to act as a metabolic stressor attending disease progression. The fish-derived omega-3 fatty acids, eicosapentaenoic acid and docosahexaenoic acid, have been examined in clinical trials of Huntington disease patients. Drugs that combat the dysregulated lipid milieu in Huntington disease may help treat this perplexing and catastrophic genetic disease.

  3. Arithmetic Word-Problem-Solving in Huntington's Disease

    Science.gov (United States)

    Allain, P.; Verny, C.; Aubin, G.; Pinon, K.; Bonneau, D.; Dubas, F.; Gall, D.L.

    2005-01-01

    The purpose of this study was to examine executive functioning in patients with Huntington's disease using an arithmetic word-problem-solving task including eight solvable problems of increasing complexity and four aberrant problems. Ten patients with Huntington's disease and 12 normal control subjects matched by age and education were tested.…

  4. Comprehension of Complex Discourse in Different Stages of Huntington's Disease

    Science.gov (United States)

    Saldert, Charlotta; Fors, Angelika; Stroberg, Sofia; Hartelius, Lena

    2010-01-01

    Background: Huntington's disease not only affects motor speech control, but also may have an impact on the ability to produce and understand language in communication. Aims: The ability to comprehend basic and complex discourse was investigated in three different stages of Huntington's disease. Methods & Procedures: In this experimental group…

  5. Water resources data, Indiana, water year 2001

    Science.gov (United States)

    Stewart, James A.; Keeton, Charles R.; Hammil, Lowell E.; Nguyen, Hieu T.; Majors, Deborah K.

    2002-01-01

    Water resources data for the 2001 water year for Indiana consists of records of discharge, stage, and water quality of streams and wells; reservoir stage and contents; and water levels in lakes and wells. This report contains records of discharge for 163 stream-gaging stations, stage for 8 stream stations, stage and contents for 1 reservoir, water quality for 1 stream, water temperature at 11 sites, sediment analysis for 1 stream, water levels for 78 lakes and 88 observation wells. Also included are records of miscellaneous discharge measurements, miscellaneous levels and miscellaneous water-quality, not part of the systematic data-collection program. Data contained in this report represent that part of the National Water Data System operated by the U.S. Geological Survey in Indiana in cooperation with State and Federal agencies.

  6. Water resources data, Indiana, water year 2000

    Science.gov (United States)

    Stewart, James A.; Keeton, Charles R.; Hammil, Lowell E.; Nguyen, Hieu T.; Majors, Deborah K.

    2001-01-01

    Water resource data for the 2000 water year for Indiana consists of records of discharge, stage, and water quality of streams and wells; reservoir stage and contents; and water levels in lakes and wells. This report contains records of discharge for 166 stream-gaging stations, stage for 7 stream stations, stage and contents for 1 reservoir, water quality for 2 streams, sediment analysis for 1 stream, water levels for 79 lakes and 89 observation wells. Also included are records of miscellaneous discharge measurements, miscellaneous levels and miscellaneous water-quality, not part of the systematic data-collection program. Data contained in this report represent that part of the the National Water Data System operated by the U.S. Geological Survey in Indiana in cooperation with State and Federal agencies.

  7. Examination of Huntington's disease in a Chinese family.

    Science.gov (United States)

    Yu, Mingxia; Li, Xiaogai; Wu, Sanyun; Shen, Ji; Tu, Jiancheng

    2014-02-15

    We report brain imaging and genetic diagnosis in a family from Wuhan, China, with a history of Huntington's disease. Among 17 family members across three generations, four patients (II2, II6, III5, and III9) show typical Huntington's disease, involuntary dance-like movements. Magnetic resonance imaging found lateral ventricular atrophy in three members (II2, II6, and III5). Moreover, genetic analysis identified abnormally amplified CAG sequence repeats (> 40) in two members (III5 and III9). Among borderline cases, with clinical symptoms and brain imaging features of Huntington's disease, two cases were identified (II2 and II6), but shown by mutation analysis for CAG expansions in the important transcript 15 gene, to be non-Huntington's disease. Our findings suggest that clinical diagnosis of Huntington's disease requires a combination of clinical symptoms, radiological changes, and genetic diagnosis.

  8. Why Indiana Voucher Parents Choose Private Schools

    Science.gov (United States)

    Friedman Foundation for Educational Choice, 2014

    2014-01-01

    In 2011, Indiana enacted the broadest school voucher program in the country. In the program's first year, almost 4,000 families used vouchers to attend participating private schools, with the number of students applying for vouchers more than doubling in each of the following two years, to 9,324 students in 2012-13, and 19,809 in 2013-14. The…

  9. Indiana Wesleyan University SPS Physics Outreach to Rural Middle School and High School Students

    Science.gov (United States)

    Ostrander, Joshua; Rose, Heath; Burchell, Robert; Ramos, Roberto

    2013-03-01

    The Society of Physics Students chapter at Indiana Wesleyan University is unusual in that it has no physics major, only physics minors. Yet while just over a year old, IWU-SPS has been active in performing physics outreach to middle school and high school students, and the rural community of Grant County. Our year-old SPS chapter consists of majors from Chemistry, Nursing, Biology, Exercise Science, Computer Science, Psychology, Pastoral Studies, and Science Education, who share a common interest in physics and service to the community. IWU currently has a physics minor and is currently working to build a physics major program. Despite the intrinsic challenges, our multi-disciplinary group has been successful at using physics demonstration equipment and hands-on activities and their universal appeal to raise the interest in physics in Grant County. We report our experience, challenges, and successes with physics outreach. We describe in detail our two-pronged approach: raising the level of physics appreciation among the IWU student community and among pre-college students in a rural community of Indiana. Acknowledgements: We acknowledge the support of the Society of Physics Students through a Marsh White Outreach Award and a Blake Lilly Prize.

  10. The Indiana Climate Change Impacts Assessment

    Science.gov (United States)

    Dukes, J. S.; Widhalm, M.

    2016-12-01

    With coordination from the Purdue Climate Change Research Center, experts and stakeholders from across Indiana are working together to develop a state-focused assessment to inform decision makers, policy makers, and interested citizens about the likely impacts of climate change in Indiana. While this assessment is not intended to provide policy recommendations, we anticipate it will elevate conversations about climate change risks within a state that is not traditionally focused on these issues, and provide the baseline data needed for moving forward with improved planning and actions. Our guiding principal throughout this process is creating information that matters. We are connecting with stakeholders before, during, and after the assessment process to understand key vulnerabilities, risks, and reasons for concern so we can ensure the Indiana Climate Change Impacts Assessment (IN CCIA) includes relevant information that is usable by state and local decision makers.The IN CCIA is building a statewide network of experts and stakeholders interested in climate change that can serve as a foundation for a sustained assessment process. This presentation will describe the grassroots, collaborative approach being followed as we conduct this assessment, and discuss the opportunities and challenges encountered along the way.

  11. Residual CO2 trapping in Indiana limestone.

    Science.gov (United States)

    El-Maghraby, Rehab M; Blunt, Martin J

    2013-01-01

    We performed core flooding experiments on Indiana limestone using the porous plate method to measure the amount of trapped CO(2) at a temperature of 50 °C and two pressures: 4.2 and 9 MPa. Brine was mixed with CO(2) for equilibration, then the mixture was circulated through a sacrificial core. Porosity and permeability tests conducted before and after 884 h of continuous core flooding confirmed negligible dissolution. A trapping curve for supercritical (sc)CO(2) in Indiana showing the relationship between the initial and residual CO(2) saturations was measured and compared with that of gaseous CO(2). The results were also compared with scCO(2) trapping in Berea sandstone at the same conditions. A scCO(2) residual trapping end point of 23.7% was observed, indicating slightly less trapping of scCO(2) in Indiana carbonates than in Berea sandstone. There is less trapping for gaseous CO(2) (end point of 18.8%). The system appears to be more water-wet under scCO(2) conditions, which is different from the trend observed in Berea; we hypothesize that this is due to the greater concentration of Ca(2+) in brine at higher pressure. Our work indicates that capillary trapping could contribute to the immobilization of CO(2) in carbonate aquifers.

  12. Communication and Huntington's Disease: Qualitative Interviews and Focus Groups with Persons with Huntington's Disease, Family Members, and Carers

    Science.gov (United States)

    Hartelius, Lena; Jonsson, Maria; Rickeberg, Anneli; Laakso, Katja

    2010-01-01

    Background: As an effect of the cognitive, emotional and motor symptoms associated with Huntington's disease, communicative interaction is often dramatically changed. No study has previously included the subjective reports on this subject from individuals with Huntington's disease. Aims: To explore the qualitative aspects of how communication is…

  13. 3-NP-induced neurodegeneration studies in experimental models of Huntington's disease : apoptosis in Huntington's disease

    NARCIS (Netherlands)

    Vis, Johanna Catharina

    2005-01-01

    This thesis investigates the possible role of apoptosis, or programmed cell death, in Huntington's disease (HD). HD is caused by an expanded CAG repeat in the N-terminal region of the huntingtin protein leading to specific neostriatal neurodegeneration. The sequence of events that leads to this sele

  14. Hydrologic data and groundwater flow simulations in the vicinity of Long Lake, Indiana Dunes National Lakeshore, near Gary, Indiana

    Science.gov (United States)

    Lampe, David C.; Bayless, E. Randall

    2013-01-01

    The U.S. Geological Survey (USGS) collected data and simulated groundwater flow to increase understanding of the hydrology and the effects of drainage alterations to the water table in the vicinity of Long Lake, near Gary, Indiana. East Long Lake and West Long Lake (collectively known as Long Lake) make up one of the largest interdunal lakes within the Indiana Dunes National Lakeshore. The National Park Service is tasked with preservation and restoration of wetlands in the Indiana Dunes National Lakeshore along the southern shoreline of Lake Michigan. Urban development and engineering have modified drainage and caused changes in the distribution of open water, streams and ditches, and groundwater abundance and flow paths. A better understanding of the effects these modifications have on the hydrologic system in the area will help the National Park Service, the Gary Sanitary District (GSD), and local stakeholders manage and protect the resources within the study area. This study used hydrologic data and steady-state groundwater simulations to estimate directions of groundwater flow and the effects of various engineering controls and climatic conditions on the hydrology near Long Lake. Periods of relatively high and low groundwater levels were examined and simulated by using MODFLOW and companion software. Simulated hydrologic modifications examined the effects of (1) removing the beaver dams in US-12 ditch, (2) discontinuing seepage of water from the filtration pond east of East Long Lake, (3) discontinuing discharge from US-12 ditch to the GSD sewer system, (4) decreasing discharge from US-12 ditch to the GSD sewer system, (5) connecting East Long Lake and West Long Lake, (6) deepening County Line Road ditch, and (7) raising and lowering the water level of Lake Michigan. Results from collected hydrologic data indicate that East Long Lake functioned as an area of groundwater recharge during October 2002 and a “flow-through” lake during March 2011, with the

  15. Characterization of conservative somatic instability of the CAG repeat region in Huntington`s disease

    Energy Technology Data Exchange (ETDEWEB)

    Schaefer, F.V.; Calikoglu, A.S.; Whetsell, L.H. [H.A. Chapman Research Institute of Medical Genetics, Tulsa, OK (United States)

    1994-09-01

    Instability and enlargement of a CAG repeat region at the beginning of the huntingtin gene (IT-15) has been linked with Huntington`s disease. The CAG repeat size shows a highly significant correlation with age-of-onset of clinicial features in individuals with 40 or more repeats who have Huntington disease. The clinical status of nonsymptomatic individuals with 30 to 39 CAG repeats is considered ambiguous. In order to define more carefully the nature of the HD expansion instability, we examined patients in our HD population using a discriminating fluorescence-based PCR approach. The degree of somatic mutation increases with both earlier age of onset and the size of the inherited allele. A single prominent band one repeat larger than the index peak was typical in individuals with 40-41 CAG repeats. Three to four larger bands are typically discerned in individuals with 50 or more repeats. In an extreme example, an individual with approximately 95 repeats had at least 8 prominent bands. Plotting the degree of somatic mutation relative to the size of the HD allele shows somatic mutation activity increases with size. By this approach 40-60% of the alleles in a 40-41 CAG repeat HD loci is represented in the primary allele. In contrast, the primary allele represents a relatively minor proportion of the total alleles for expansions greater than 50 CAG repeats (10-20%). The limited range of somatic mutation suggest that the instability is restricted to very early stages of embryogenesis before tissue development diverges or that persistent somatic instability occurs at a slow rate. Therefore, the properties of somatic instability in Huntington`s disease have aspects that are both in common but also different from that found in other trinucleotide repeat expanding diseases such as myotonic muscular dystrophy and fragile X syndrome.

  16. Altered Fractional Anisotropy in Early Huntington's Disease

    Directory of Open Access Journals (Sweden)

    Silky Singh

    2013-02-01

    Full Text Available Huntington's disease (HD is a dominantly inherited neurodegenerative disease best known for chorea. The disorder includes numerous other clinical features including mood disorder, eye movement abnormalities, cognitive disturbance, pendular knee reflexes, motor impersistence, and postural instability. We describe a mild case of HD early in the disease course with depression and subtle neurological manifestations. In addition, we review MRI and diffusion tensor imaging features in this patient. The bicaudate ratio, a measure of caudate atrophy, was increased. Fractional anisotropy values of the bilateral caudate and putamen were increased, signifying neurodegeneration of these structures in HD.

  17. Huntingtin processing in pathogenesis of Huntington disease

    Institute of Scientific and Technical Information of China (English)

    Zhenghong QIN; Zhenlun GU

    2004-01-01

    Huntington's disease (HD) is caused by an expansion of the polyglutamine tract in the protein named huntingtin.The expansion of polyglutamine tract induces selective degeneration of striatal projection neurons and cortical pyramidal neurons. The bio-hallmark of HD is the formation of intranuclear inclusions and cytoplasmic aggregates in association with other cellular proteins in vulnerable neurons. Accumulation of N-terminal mutant huntingtin in HD brains is prominent. These pathological features are related to protein misfolding and impairments in protein processing and degradation in neurons. This review focused on the role of proteases in huntingtin cleavage and degradation and the contribution of altered processing of mutant huntingtin to HD pathogenesis.

  18. Cystathionine γ-lyase deficiency mediates neurodegeneration in Huntington's disease.

    Science.gov (United States)

    Paul, Bindu D; Sbodio, Juan I; Xu, Risheng; Vandiver, M Scott; Cha, Jiyoung Y; Snowman, Adele M; Snyder, Solomon H

    2014-05-01

    Huntington's disease is an autosomal dominant disease associated with a mutation in the gene encoding huntingtin (Htt) leading to expanded polyglutamine repeats of mutant Htt (mHtt) that elicit oxidative stress, neurotoxicity, and motor and behavioural changes. Huntington's disease is characterized by highly selective and profound damage to the corpus striatum, which regulates motor function. Striatal selectivity of Huntington's disease may reflect the striatally selective small G protein Rhes binding to mHtt and enhancing its neurotoxicity. Specific molecular mechanisms by which mHtt elicits neurodegeneration have been hard to determine. Here we show a major depletion of cystathionine γ-lyase (CSE), the biosynthetic enzyme for cysteine, in Huntington's disease tissues, which may mediate Huntington's disease pathophysiology. The defect occurs at the transcriptional level and seems to reflect influences of mHtt on specificity protein 1, a transcriptional activator for CSE. Consistent with the notion of loss of CSE as a pathogenic mechanism, supplementation with cysteine reverses abnormalities in cultures of Huntington's disease tissues and in intact mouse models of Huntington's disease, suggesting therapeutic potential.

  19. Chicago, Indiana set for "world's largest scientific experiment"

    CERN Multimedia

    2007-01-01

    "The mission of The Hoosier Coefficient, which appears on MidestBusiness.com eveery Thursday, is to profile the often-overlooked rich technology development and commercialization in Indiana. The Hoosier state is home to four of the top technology research and engineering universities in the nation and tech pros ignore Indiana at their own peril." (1 page)

  20. Indiana Emergent Bilingual Student Time to Reclassification: A Survival Analysis

    Science.gov (United States)

    Burke, April M.; Morita-Mullaney, Trish; Singh, Malkeet

    2016-01-01

    In this study, we employed a discrete-time survival analysis model to examine Indiana emergent bilingual time to reclassification as fluent English proficient. The data consisted of five years of statewide English language proficiency scores. Indiana has a large and rapidly growing Spanish-speaking emergent bilingual population, and these students…

  1. Mobility of Graduates from Indiana Public Postsecondary Education. Working Paper.

    Science.gov (United States)

    Indiana State Commission for Higher Education, Indianapolis.

    This report addresses the extent to which graduates of Indiana's colleges and universities remain in Indiana, as opposed to moving to another state. Using public institution data from the Commission's Student Information System (SIS) and Driver's License data from the Bureau of Motor Vehicles (BMV), staff tracked the 1990-91 degree recipients to…

  2. 2005 Public Opinion Survey on Education in Indiana

    Science.gov (United States)

    Plucker, Jonathan A.; Spradlin, Terry E.; Zapf, Jason S.; Chien, Rosanne W.; Jackson, Rose A.

    2006-01-01

    The Public Opinion Survey on Education in Indiana is a longitudinal effort to identify and monitor Indiana residents' attitudes toward and perceptions of public education issues. The study reports public opinion on issues of major importance concerning public schools and K-12 education policy. The Benchmark Survey was conducted in November 2003…

  3. 76 FR 44613 - Designation of Eight Counties as High Intensity Drug Trafficking Areas

    Science.gov (United States)

    2011-07-26

    ...The Director of the Office of National Drug Control Policy has designated eight additional counties as High Intensity Drug Trafficking Areas pursuant to 21 U.S.C. 1706. The new counties are (1) Orange County in New York as part of the New York/New Jersey HIDTA; (2) Medocino County in California as part of the Northern California HIDTA; (3) Porter County in Indiana as part of the Lake County HIDTA; (4) Lexington and Richland Counties in South Carolina as part of the Atlanta HIDTA; (5) Harford County in Maryland as part of the Washington/Baltimore HIDTA; (6) Putnam and Mercer Counties in West Virginia as part of the Appalachia HIDTA.

  4. Unawareness of motor phenoconversion in Huntington disease.

    Science.gov (United States)

    McCusker, Elizabeth A; Gunn, David G; Epping, Eric A; Loy, Clement T; Radford, Kylie; Griffith, Jane; Mills, James A; Long, Jeffrey D; Paulsen, Jane S

    2013-09-24

    To determine whether Huntington disease (HD) mutation carriers have motor symptoms (complaints) when definite motor onset (motor phenoconversion) is diagnosed and document differences between the groups with and without unawareness of motor signs. We analyzed data from 550 HD mutation carriers participating in the multicenter PREDICT-HD Study followed through the HD prodrome. Data analysis included demographics, the Unified Huntington's Disease Rating Scale (UHDRS) and the Participant HD History of symptoms, self-report of progression, and cognitive, behavioral, and imaging measures. Unawareness was identified when no motor symptoms were self-reported but when definite motor HD was diagnosed. Of 38 (6.91%) with onset of motor HD, almost half (18/38 = 47.36%) had no motor symptoms despite signs of disease on the UHDRS motor rating and consistent with unawareness. A group with motor symptoms and signs was similar on a range of measures to the unaware group. Those with unawareness of HD signs reported less depression. Patients with symptoms had more striatal atrophy on imaging measures. Only half of the patients with newly diagnosed motor HD had motor symptoms. Unaware patients were less likely to be depressed. Self-report of symptoms may be inaccurate in HD at the earliest stage.

  5. High Protein Diet and Huntington's Disease.

    Directory of Open Access Journals (Sweden)

    Chiung-Mei Chen

    Full Text Available Huntington's disease (HD is a neurodegenerative disorder caused by the huntingtin (HTT gene with expanded CAG repeats. In addition to the apparent brain abnormalities, impairments also occur in peripheral tissues. We previously reported that mutant Huntingtin (mHTT exists in the liver and causes urea cycle deficiency. A low protein diet (17% restores urea cycle activity and ameliorates symptoms in HD model mice. It remains unknown whether the dietary protein content should be monitored closely in HD patients because the normal protein consumption is lower in humans (~15% of total calories than in mice (~22%. We assessed whether dietary protein content affects the urea cycle in HD patients. Thirty HD patients were hospitalized and received a standard protein diet (13.7% protein for 5 days, followed by a high protein diet (HPD, 26.3% protein for another 5 days. Urea cycle deficiency was monitored by the blood levels of citrulline and ammonia. HD progression was determined by the Unified Huntington's Disease Rating Scale (UHDRS. The HPD increased blood citrulline concentration from 15.19 μmol/l to 16.30 μmol/l (p = 0.0378 in HD patients but did not change blood ammonia concentration. A 2-year pilot study of 14 HD patients found no significant correlation between blood citrulline concentration and HD progression. Our results indicated a short period of the HPD did not markedly compromise urea cycle function. Blood citrulline concentration is not a reliable biomarker of HD progression.

  6. Indiana Regional Transfer Study: The Student Experience of Transfer Pathways between Ivy Tech Community College and Indiana University

    Science.gov (United States)

    Kadlec, Alison; Gupta, Jyoti

    2014-01-01

    This report details findings from focus groups with college students across Indiana. All of these students were planning to transfer or had transferred from the state community college system, Ivy Tech, to a school in the Indiana University system. We wanted to find out what these students had to say about their experiences preparing for and…

  7. Analyzing the attributes of Indiana's STEM schools

    Science.gov (United States)

    Eltz, Jeremy

    "Primary and secondary schools do not seem able to produce enough students with the interest, motivation, knowledge, and skills they will need to compete and prosper in the emerging world" (National Academy of Sciences [NAS], 2007a, p. 94). This quote indicated that there are changing expectations for today's students which have ultimately led to new models of education, such as charters, online and blended programs, career and technical centers, and for the purposes of this research, STEM schools. STEM education as defined in this study is a non-traditional model of teaching and learning intended to "equip them [students] with critical thinking, problem solving, creative and collaborative skills, and ultimately establishes connections between the school, work place, community and the global economy" (Science Foundation Arizona, 2014, p. 1). Focusing on science, technology, engineering, and math (STEM) education is believed by many educational stakeholders to be the solution for the deficits many students hold as they move on to college and careers. The National Governors Association (NGA; 2011) believes that building STEM skills in the nation's students will lead to the ability to compete globally with a new workforce that has the capacity to innovate and will in turn spur economic growth. In order to accomplish the STEM model of education, a group of educators and business leaders from Indiana developed a comprehensive plan for STEM education as an option for schools to use in order to close this gap. This plan has been promoted by the Indiana Department of Education (IDOE, 2014a) with the goal of increasing STEM schools throughout Indiana. To determine what Indiana's elementary STEM schools are doing, this study analyzed two of the elementary schools that were certified STEM by the IDOE. This qualitative case study described the findings and themes from two elementary STEM schools. Specifically, the research looked at the vital components to accomplish STEM

  8. Association of Huntington's disease and schizophrenia-like psychosis in a Huntington's disease pedigree

    OpenAIRE

    Guimarães João; Xavier Miguel; Corrêa Bernardo

    2006-01-01

    Abstract Background Huntington's disease (HD) is a dominantly inherited, neurodegenerative disorder due to expansion of a polymorphic trinucleotide repeat in the short arm of chromosome 4. Clinical manifestations consist of a triad of choreic movements, cognitive decline and psychiatric syndromes starting in the fourth to fifth decade. Psychiatric manifestations vary and may precede motor and cognitive changes. Personality changes and depression occur most commonly. Paranoid schizophrenia-lik...

  9. O desenvolvimento político em Huntington e Fukuyama Huntington and Fukuyama on political development

    Directory of Open Access Journals (Sweden)

    Natália Nóbrega de Mello

    2010-01-01

    Full Text Available O artigo contrasta as teses de Huntington e Fukuyama sobre desenvolvimento político. As obras analisadas, Ordem política nas sociedades em mudança e O fim da história, inscrevem-se entre duas conjunturas decisivas - 1968 e 1989. Huntington desmontou a equivalência entre desenvolvimento político e modernização e Fukuyama reafirmou a democracia como o destino de todos os países e, desse modo, como o fim da história. Nesta comparação, dois eixos se sobressaem: o contexto de produção das obras e a alternância entre os polos teóricos da democracia e da estabilidade. Procura-se demonstrar como, apesar de reinserir a democracia no desenvolvimento político, a instabilidade continua a ser um foco privilegiado de análise no pensamento de Fukuyama.The article contrasts the theories of Huntington and Fukuyama on political development. The analyzed works, Political order in changing societies and The end of history, fall between two decisive historical moments - in 1968 and 1989. Huntington disassembled the equivalence between political development and modernization; Fukuyama reaffirmed democracy as the destiny of all countries and, as such, it is the end of history. In this comparison, two axes call our attention: the production context of these works and the alternation between the theoreticals poles of democracy and stability. The article shows how, although reenters democracy in the political development theory, instablility remains a prime focus of analysis in Fukuyama's thought.

  10. Geology, Bedrock - BEDROCK_GEOLOGY_RGM_250K_IN: Bedrock geology of Indiana, from the Regional Geologic Map Series of the Indiana Geological Survey (Indiana Geological Survey, 1:250,000, Polygon Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — BEDROCK_GEOL_RGM_IN is a polygon shapefile that shows the bedrock geology of the state of Indiana, produced from the Indiana Geological Survey Regional Geologic Map...

  11. Américo Negrette and Huntington's disease

    Directory of Open Access Journals (Sweden)

    Mariana Moscovich

    2011-08-01

    Full Text Available The authors present a historical review of the seminal clinical contribution of Professor Américo Negrette, a Venezuelan neurologist, to the evolution of scientific knowledge about Huntington's disease.

  12. 1H magnetic resonance spectroscopy in preclinical Huntington disease

    NARCIS (Netherlands)

    van Oostrom, Joost C. H.; Sijens, Paul E.; Roos, Raymund A. C.; Leenders, Klaus L.

    2007-01-01

    Huntington disease (HD) is a hereditary brain disease, causing progressive deterioration after a preclinical phase. The pathophysiology of early brain abnormalities around disease onset is largely unknown. Some preclinical mutation carriers (PMC) show structural or metabolic changes on brain imaging

  13. Juvenile Huntington's disease: a case report and literature review.

    Science.gov (United States)

    Reyes Molón, L; Yáñez Sáez, R M; López-Ibor Alcocer, M I

    2010-01-01

    Huntington's disease is the most frequent neurodegenerative disease with a prevalence of fewer than 10 cases per 10,000 inhabitants; the juvenile form is responsible for less than 10% of all cases. Huntington's disease belongs to the group known as "triad syndromes," which evolve with cognitive, motor and neuropsychiatric manifestations. Around 30% of patients debut with behavioral symptoms, which are a major challenge for management by patients, families, and caregivers. Huntington's disease (HD) is reviewed and a case of juvenile onset is reported in this article. The characteristics of juvenile-onset Huntington's disease (HD) differ from those of adult-onset HD, as chorea does not occur, although bradykinesia, dystonia, and signs of cerebellar disorder, such as rigidity, are present, frequently in association with convulsive episodes and psychotic manifestations.

  14. Genetics Home Reference: Huntington disease-like syndrome

    Science.gov (United States)

    ... 21915. Citation on PubMed Wild EJ, Tabrizi SJ. Huntington's disease phenocopy syndromes. Curr Opin Neurol. 2007 Dec;20(6):681-7. Review. Citation on PubMed Reviewed : August 2008 Published : August ...

  15. Episodic Memory Decline in Huntington's Disease, A Binding Deficit?

    NARCIS (Netherlands)

    El Haj, M.; Caillaud, M.; Fasotti, L.; Verny, C.; Allain, P.

    2013-01-01

    Background: Huntington's disease (HD) is characterized by episodic memory deterioration. Objective: Our paper investigates the cognitive mechanisms that might underlie this decline. To this aim, we tested two executive hypotheses, the binding and the inhibition hypotheses. Methods: Fifteen HD patien

  16. O paradigma de Huntington e o realismo político Huntington's paradigm and political realism

    Directory of Open Access Journals (Sweden)

    José R. Novaes Chiappin

    1994-12-01

    Full Text Available Examina-se a proposta de Huntington de um novo paradigma da política internacional (centrado na idéia de "civilizações" em substituição ao paradigma do realismo. Demonstra-se que se trata, na realidade, de um subparadigma do realismo e, portanto, a ele subordinado. Aplica-se isso à mudança da concepção estratégica de "contenção", que passa a aplicar-se às civilizações não-ocidentais e não mais ao expansionismo soviético.Huntington's proposal of a new paradigm for international politics (focused on the idea of "civilizations", meant to replace the paradigm of realism, is examined. It is shown that the proposed new paradigm should in fact be viewed as as sub-paradigm of the realist one. In particular, it is pointed out that Huntington's proposal, in a realist vein, draws on the idea of "containment", which is now directed (instead of its former target, the soviet expansionism to non-Western civilizations.

  17. Subtle changes among presymptomatic carriers of the Huntington's disease gene

    OpenAIRE

    S. Kirkwood; Siemers, E.; Hodes, M; Conneally, P; Christian, J.; Foroud, T

    2000-01-01

    OBJECTIVES—To compare the neurological and psychometric characteristics of presymptomatic gene carriers and non-gene carriers who are at risk for developing Huntington's disease so as to characterise early signs of disease and to identify markers of neurological function that could be used to assess the impact of experimental therapies on the progression of disease, even among those who are clinically presymptomatic.
METHODS—A sample of people at risk for Huntington's dis...

  18. Samuel Huntington, Clash of Civilizations: A Book Review

    OpenAIRE

    Yrd. Doç. Dr. Cengiz Kartýn

    2015-01-01

    Samuel Huntington's The Clash of Civilizations was written in 1993 by him. Study is a work containing the article and the responses to this article. Work is composed of two main parts. Makes the important point of this study is the process that began with the September 11 attacks by some strategists that is exactly the way towards a world where it is hidden in Huntington's fictionalized articulate.

  19. Linking SNPs to CAG repeat length in Huntington's disease patients.

    Science.gov (United States)

    Liu, Wanzhao; Kennington, Lori A; Rosas, H Diana; Hersch, Steven; Cha, Jang-Ho; Zamore, Phillip D; Aronin, Neil

    2008-11-01

    Allele-specific silencing using small interfering RNAs targeting heterozygous single-nucleotide polymorphisms (SNPs) is a promising therapy for human trinucleotide repeat diseases such as Huntington's disease. Linking SNP identities to the two HTT alleles, normal and disease-causing, is a prerequisite for allele-specific RNA interference. Here we describe a method, SNP linkage by circularization (SLiC), to identify linkage between CAG repeat length and nucleotide identity of heterozygous SNPs using Huntington's disease patient peripheral blood samples.

  20. Neuropathological diagnosis and CAG repeat expansion in Huntington's disease.

    OpenAIRE

    Xuereb, J H; MacMillan, J C; Snell, R; Davies, P.; Harper, P S

    1996-01-01

    OBJECTIVE--To correlate the degree of CAG repeat expansion with neuropathological findings in Huntington's disease. METHODS--The CAG repeat polymorphism was analysed in a large series of brain samples from 268 patients with a clinical diagnosis of Huntington's disease in which full neuropathological data was available. RESULTS--Analysis by polymerase chain reaction was successful in 63% of samples (169 of 268). Repeat expansions were detected in 152 of 153 (99%) samples with a neuropathologic...

  1. Levodopa responsive parkinsonism in an adult with Huntington's disease

    OpenAIRE

    Racette, B.; Perlmutter, J

    1998-01-01

    A patient is reported on with Huntington's disease who, as an adult, first developed severe parkinsonism with bradykinesia, rigidity, postural instability and festinating gait. His clinical signs were similar to those of the Westphal variant of Huntington's disease except that he also had resting tremor and a supranuclear gaze palsy. Magnetic resonance imaging showed caudate and putamen atrophy. Genetic analysis disclosed 49 triple CAG repeats in allele 1 and 17 in allele 2 ...

  2. Striatal grafts in a rat model of Huntington's disease

    DEFF Research Database (Denmark)

    Guzman, R; Meyer, M; Lövblad, K O;

    1999-01-01

    Survival and integration into the host brain of grafted tissue are crucial factors in neurotransplantation approaches. The present study explored the feasibility of using a clinical MR scanner to study striatal graft development in a rat model of Huntington's disease. Rat fetal lateral ganglionic...... eminences grown as free-floating roller-tube cultures can be successfully grafted in a rat Huntington model and that a clinical MR scanner offers a useful noninvasive tool for studying striatal graft development....

  3. [Olanzapine improves chorea in patients with Huntington's disease].

    Science.gov (United States)

    Jiménez-Jiménez, F J; de Toledo, M; Puertas, I; Barón, M; Zurdo, M; Barcenilla, B

    The main treatment for choreatic movements associated to Huntington s disease are the neuroleptic drugs, however, its use causes long term troubles. We describe two patients with a predominantly choreic Huntington s disease, who experience improvement of choreatic movements after introduction of olanzapine to their treatment, being this drug well tolerated. The improvement of chorea suggests that olanzapine has a dopaminergic D2 receptors blocking action.

  4. A case report of juvenile Huntington disease

    Directory of Open Access Journals (Sweden)

    Anita Choudhary

    2017-09-01

    Full Text Available Huntington disease (HD is a progressive neurodegenerative disorder, characterized by autosomal dominant inheritance, movement disorder, dementia, and behavioural disturbances. It is caused by a mutation in IT15 gene on chromosome 4p16.3, which leads to unstable CAG trinucleotide repeat expansion. The onset of juvenile HD occurs before the 2nd decade of life and comprises approximately 10% of total HD patients. Juvenile HD differs in symptomatology and is usually transmitted from paternal side with genetic anticipation phenomenon. Magnetic resonance imaging (MRI of the brain shows specific changes of early affection of caudate nucleus and putamen. Multidisciplinary approach with symptomatic treatment of specific symptoms is the current available management. Gene editing and gene silencing treatment are under trial. Hereby, we introduce a case of an 8-year-old boy, who presented with typical symptoms of juvenile HD, positive family history with genetic anticipation phenomenon and characteristic MRI findings.

  5. Plants and phytochemicals for Huntington's disease.

    Science.gov (United States)

    Choudhary, Sunayna; Kumar, Puneet; Malik, Jai

    2013-07-01

    Huntington's disease (HD) is a neurodegenerative disorder characterized by progressive motor dysfunction, including chorea and dystonia, emotional disturbances, memory, and weight loss. The medium spiny neurons of striatum and cortex are mainly effected in HD. Various hypotheses, including molecular genetics, oxidative stress, excitotoxicity, metabolic dysfunction, and mitochondrial impairment have been proposed to explain the pathogenesis of neuronal dysfunction and cell death. Despite no treatment is available to fully stop the progression of the disease, there are treatments available to help control the chorea. The present review deals with brief pathophysiology of the disease, plants and phytochemicals that have shown beneficial effects against HD like symptoms. The literature for the current review was collected using various databases such as Science direct, Pubmed, Scopus, Sci-finder, Google Scholar, and Cochrane database with a defined search strategy.

  6. The choreography of neuroinflammation in Huntington's disease.

    Science.gov (United States)

    Crotti, Andrea; Glass, Christopher K

    2015-06-01

    Currently, the concept of 'neuroinflammation' includes inflammation associated with neurodegenerative diseases, in which there is little or no infiltration of blood-derived immune cells into the brain. The roles of brain-resident and peripheral immune cells in these inflammatory settings are poorly understood, and it is unclear whether neuroinflammation results from immune reaction to neuronal dysfunction/degeneration, and/or represents cell-autonomous phenotypes of dysfunctional immune cells. Here, we review recent studies examining these questions in the context of Huntington's disease (HD), where mutant Huntingtin (HTT) is expressed in both neurons and glia. Insights into the cellular and molecular mechanisms underlying neuroinflammation in HD may provide a better understanding of inflammation in more complex neurodegenerative disorders, and of the contribution of the neuroinflammatory component to neurodegenerative disease pathogenesis.

  7. Huntington disease: DNA analysis in brazilian population

    Directory of Open Access Journals (Sweden)

    RASKIN SALMO

    2000-01-01

    Full Text Available Huntington disease (HD is associated with expansions of a CAG trinucleotide repeat in the HD gene. Accurate measurement of a specific CAG repeat sequence in the HD gene in 92 Brazilian controls without HD, 44 Brazilian subjects with clinical findings suggestive of HD and 40 individuals from 6 putative HD families, showed a range from 7 to 33 repeats in normal subjects and 39 to 88 repeats in affected subjects. A trend between early age at onset of first symptoms and increasing number of repeats was seen. Major increase of repeat size through paternal inheritance than through maternal inheritance was observed. Data generated from this study may have significant implications for the etiology, knowledge of the incidence, diagnosis, prognosis, genetic counseling and treatment of HD Brazilian patients.

  8. Huntington's Disease: Pathogenic Mechanisms and Therapeutic Targets.

    Science.gov (United States)

    Wright, Dean J; Renoir, Thibault; Gray, Laura J; Hannan, Anthony J

    2017-01-01

    Huntington's disease (HD) is a tandem repeat disorder involving neurodegeneration and a complex combination of symptoms. These include psychiatric symptoms, cognitive deficits culminating in dementia, and the movement disorder epitomised by motor signs such as chorea. HD is caused by a CAG repeat expansion encoding an extended tract of the amino acid glutamine in the huntingtin protein. This polyglutamine expansion appears to induce a 'change of function', possibly a 'gain of function', in the huntingtin protein, which leads to various molecular and cellular cascades of pathogenesis. In the current review, we will briefly describe these broader aspects of HD pathogenesis, but will then focus on specific aspects where there are substantial bodies of experimental evidence, including oxidative stress, mitochondrial dysfunction, glutamatergic dysfunction and neuroinflammation. Furthermore, we will review recent preclinical therapeutic approaches targeting some of these pathogenic pathways, their clinical implications and future directions.

  9. Huntington's Disease: Relationship Between Phenotype and Genotype.

    Science.gov (United States)

    Sun, Yi-Min; Zhang, Yan-Bin; Wu, Zhi-Ying

    2017-01-01

    Huntington's disease (HD) is an autosomal dominant inherited neurodegenerative disease with the typical manifestations of involuntary movements, psychiatric and behavior disorders, and cognitive impairment. It is caused by the dynamic mutation in CAG triplet repeat number in exon 1 of huntingtin (HTT) gene. The symptoms of HD especially the age at onset are related to the genetic characteristics, both the CAG triplet repeat and the modified factors. Here, we reviewed the recent advancement on the genotype-phenotype relationship of HD, mainly focus on the characteristics of different expanded CAG repeat number, genetic modifiers, and CCG repeat number in the 3' end of CAG triplet repeat and their effects on the phenotype. We also reviewed the special forms of HD (juvenile HD, atypical onset HD, and homozygous HD) and their phenotype-genotype correlations. The review will aid clinicians to predict the onset age and disease course of HD, give the genetic counseling, and accelerate research into the HD mechanism.

  10. Huntington's Disease: Calcium Dyshomeostasis and Pathology Models.

    Science.gov (United States)

    Kolobkova, Y A; Vigont, V A; Shalygin, A V; Kaznacheyeva, E V

    2017-01-01

    Huntington's disease (HD) is a severe inherited neurodegenerative disorder characterized by motor dysfunction, cognitive decline, and mental impairment. At the molecular level, HD is caused by a mutation in the first exon of the gene encoding the huntingtin protein. The mutation results in an expanded polyglutamine tract at the N-terminus of the huntingtin protein, causing the neurodegenerative pathology. Calcium dyshomeostasis is believed to be one of the main causes of the disease, which underlies the great interest in the problem among experts in molecular physiology. Recent studies have focused on the development of animal and insect HD models, as well as patient-specific induced pluripotent stem cells (HD-iPSCs), to simulate the disease's progression. Despite a sesquicentennial history of HD studies, the issues of diagnosis and manifestation of the disease have remained topical. The present review addresses these issues.

  11. Cerebrospinal Fluid Biomarkers for Huntington's Disease.

    Science.gov (United States)

    Byrne, Lauren M; Wild, Edward J

    2016-01-01

    Cerebrospinal fluid (CSF) is enriched in brain-derived components and represents an accessible and appealing means of interrogating the CNS milieu to study neurodegenerative diseases and identify biomarkers to facilitate the development of novel therapeutics. Many such CSF biomarkers have been proposed for Huntington's disease (HD) but none has been validated for clinical trial use. Across many studies proposing dozens of biomarker candidates, there is a notable lack of statistical power, consistency, rigor and validation. Here we review proposed CSF biomarkers including neurotransmitters, transglutaminase activity, kynurenine pathway metabolites, oxidative stress markers, inflammatory markers, neuroendocrine markers, protein markers of neuronal death, proteomic approaches and mutant huntingtin protein itself. We reflect on the need for large-scale, standardized CSF collections with detailed phenotypic data to validate and qualify much-needed CSF biomarkers for clinical trial use in HD.

  12. Contribution of Neuroepigenetics to Huntington's Disease.

    Science.gov (United States)

    Francelle, Laetitia; Lotz, Caroline; Outeiro, Tiago; Brouillet, Emmanuel; Merienne, Karine

    2017-01-01

    Unbalanced epigenetic regulation is thought to contribute to the progression of several neurodegenerative diseases, including Huntington's disease (HD), a genetic disorder considered as a paradigm of epigenetic dysregulation. In this review, we attempt to address open questions regarding the role of epigenetic changes in HD, in the light of recent advances in neuroepigenetics. We particularly discuss studies using genome-wide scale approaches that provide insights into the relationship between epigenetic regulations, gene expression and neuronal activity in normal and diseased neurons, including HD neurons. We propose that cell-type specific techniques and 3D-based methods will advance knowledge of epigenome in the context of brain region vulnerability in neurodegenerative diseases. A better understanding of the mechanisms underlying epigenetic changes and of their consequences in neurodegenerative diseases is required to design therapeutic strategies more effective than current strategies based on histone deacetylase (HDAC) inhibitors. Researches in HD may play a driving role in this process.

  13. Neuronal Ca(2+) dyshomeostasis in Huntington disease.

    Science.gov (United States)

    Giacomello, Marta; Oliveros, Juan C; Naranjo, Jose R; Carafoli, Ernesto

    2013-01-01

    The expansion of the N-terminal poly-glutamine tract of the huntingtin (Htt) protein is responsible for Huntington disease (HD). A large number of studies have explored the neuronal phenotype of HD, but the molecular aethiology of the disease is still very poorly understood. This has hampered the development of an appropriate therapeutical strategy to at least alleviate its symptoms. In this short review, we have focused our attention on the alteration of a specific cellular mechanism common to all HD models, either genetic or induced by treatment with 3-NPA, i.e. the cellular dyshomeostasis of Ca(2+). We have highlighted the direct and indirect (i.e. transcriptionally mediated) effects of mutated Htt on the maintenance of the intracellular Ca(2+) balance, the correct modulation of which is fundamental to cell survival and the disturbance of which plays a key role in the death of the cell.

  14. Superfund and Toxic Release Inventory Sites - INSTITUTIONAL_CONTROLS_IDEM_IN.SHP: Institutional Control Sites in Indiana (Indiana Department of Environmental Management, Polygon Shapefile)

    Data.gov (United States)

    NSGIC State | GIS Inventory — INSTITUTIONAL_CONTROLS_IDEM_IN is a polygon shapefile that contains Institutional Control (IC) site locations in Indiana, provided by personnel of Indiana Department...

  15. Confined Feeding Operations - CONFINED_FEEDING_OPERATIONS_IDEM_IN: Confined Feeding Operation Facilities in Indiana (Indiana Department of Environmental Management, Point Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — CONFINED_FEEDING_OPERATIONS_IDEM_IN is a point shapefile that contains confined feeding operation facility locations in Indiana, provided by personnel of Indiana...

  16. Railroad Lines - RAIL_SYSTEM_ACTIVE_ABANDONED_INDOT_IN: Active and Abandoned Rail System in Indiana, 2005 (Indiana Department of Transportation, 1:1200, Line Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — RAIL_SYSTEM_ACTIVE_ABANDONED_INDOT_IN is a line shapefile that contains all active and abandoned rail lines in Indiana, provided by personnel of Indiana Department...

  17. Hazardous Material Storage Facilities and Sites - WASTE_SOLID_ACTIVE_PERMITTED_IDEM_IN: Active Permitted Solid Waste Sites in Indiana (Indiana Department of Environmental Management, Point Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — WASTE_SOLID_ACTIVE_PERMITTED_IDEM_IN is a point shapefile that contains active permitted solid waste site locations in Indiana, provided by personnel of Indiana...

  18. Superfund and Toxic Release Inventory Sites - SUPERFUND_IDEM_IN: Superfund Program Facilities in Indiana (Indiana Department of Environmental Management, Point Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — SUPERFUND_IDEM_IN is a point shapefile that contains GPS-located Superfund Program facility locations in Indiana, provided by personnel of Indiana Department of...

  19. Hazardous Material Storage Facilities and Sites - WASTE_SEPTAGE_SITES_IDEM_IN: Septage Waste Sites in Indiana (Indiana Department of Environmental Management, Point Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — WASTE_SEPTAGE_SITES_IDEM_IN is a point shapefile that contains septage waste site locations in Indiana, provided by personnel of Indiana Department of Environmental...

  20. Hazardous Material Storage Facilities and Sites - INSTITUTIONAL_CONTROLS_IDEM_IN: Institutional Control Sites in Indiana (Indiana Department of Environmental Management, Polygon Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — INSTITUTIONAL_CONTROLS_IDEM_IN is a polygon shapefile that contains Institutional Control (IC) site locations in Indiana, provided by personnel of Indiana Department...

  1. Kolm tundi päevas jõusaalis ja dieet : nii voolis Harrison Ford end taas Indiana Jonesiks / Triin Tael

    Index Scriptorium Estoniae

    Tael, Triin

    2008-01-01

    Steven Spielbergi neljas Indiana Jones'i film Harrison Fordiga nimiosas "Indiana Jones ja kristallpealuu kuningriik" ("Indiana Jones and the Kingdom of the Crystal Skull") : Ameerika Ühendriigid 2008. Indiana Jonesi tähestik

  2. Kolm tundi päevas jõusaalis ja dieet : nii voolis Harrison Ford end taas Indiana Jonesiks / Triin Tael

    Index Scriptorium Estoniae

    Tael, Triin

    2008-01-01

    Steven Spielbergi neljas Indiana Jones'i film Harrison Fordiga nimiosas "Indiana Jones ja kristallpealuu kuningriik" ("Indiana Jones and the Kingdom of the Crystal Skull") : Ameerika Ühendriigid 2008. Indiana Jonesi tähestik

  3. Indiana Bat (Myotis sodalis) Draft Recovery Plan: First Revision

    Data.gov (United States)

    US Fish and Wildlife Service, Department of the Interior — U.S. Fish and Wildlife Service's Draft recovery plan for Indiana bats, a Federally endangered species. First revision. The purpose of this draft recovery plan is to...

  4. Radiological Final Status Survey of the Hammond Depot, Hammond, Indiana

    Energy Technology Data Exchange (ETDEWEB)

    T.J. Vitkus

    2008-04-07

    ORISE conducted extensive scoping, characterization, and final status surveys of land areas and structures at the DNSC’s Hammond Depot located in Hammond, Indiana in multiple phases during 2005, 2006 and 2007.

  5. Traumatic brain injury among Indiana state prisoners.

    Science.gov (United States)

    Ray, Bradley; Sapp, Dona; Kincaid, Ashley

    2014-09-01

    Research on traumatic brain injury among inmates has focused on comparing the rate of traumatic brain injury among offenders to the general population, but also how best to screen for traumatic brain injury among this population. This study administered the short version of the Ohio State University Traumatic Brain Injury Identification Method to all male inmates admitted into Indiana state prisons were screened for a month (N = 831). Results indicate that 35.7% of the inmates reported experiencing a traumatic brain injury during their lifetime and that these inmates were more likely to have a psychiatric disorder and a prior period of incarceration than those without. Logistic regression analysis finds that a traumatic brain injury predicts the likelihood of prior incarceration net of age, race, education, and psychiatric disorder. This study suggests that brief instruments can be successfully implemented into prison screenings to help divert inmates into needed treatment.

  6. Quantitative 7T phase imaging in premanifest Huntington disease.

    Science.gov (United States)

    Apple, A C; Possin, K L; Satris, G; Johnson, E; Lupo, J M; Jakary, A; Wong, K; Kelley, D A C; Kang, G A; Sha, S J; Kramer, J H; Geschwind, M D; Nelson, S J; Hess, C P

    2014-09-01

    In vivo MR imaging and postmortem neuropathologic studies have demonstrated elevated iron concentration and atrophy within the striatum of patients with Huntington disease, implicating neuronal loss and iron accumulation in the pathogenesis of this neurodegenerative disorder. We used 7T MR imaging to determine whether quantitative phase, a measurement that reflects both iron content and tissue microstructure, is altered in subjects with premanifest Huntington disease. Local field shift, calculated from 7T MR phase images, was quantified in 13 subjects with premanifest Huntington disease and 13 age- and sex-matched controls. All participants underwent 3T and 7T MR imaging, including volumetric T1 and 7T gradient recalled-echo sequences. Local field shift maps were created from 7T phase data and registered to caudate ROIs automatically parcellated from the 3T T1 images. Huntington disease-specific disease burden and neurocognitive and motor evaluations were also performed and compared with local field shift. Subjects with premanifest Huntington disease had smaller caudate volume and higher local field shift than controls. A significant correlation between these measurements was not detected, and prediction accuracy for disease state improved with inclusion of both variables. A positive correlation between local field shift and genetic disease burden was also found, and there was a trend toward significant correlations between local field shift and neurocognitive tests of working memory and executive function. Subjects with premanifest Huntington disease exhibit differences in 7T MR imaging phase within the caudate nuclei that correlate with genetic disease burden and trend with neurocognitive assessments. Ultra-high-field MR imaging of quantitative phase may be a useful approach for monitoring neurodegeneration in premanifest Huntington disease. © 2014 by American Journal of Neuroradiology.

  7. Placebo effect characteristics observed in a single, international, longitudinal study in Huntington's disease.

    NARCIS (Netherlands)

    Cubo, E.; Gonzalez, M.; Puerto, I. del; Yebenes, J.G. de; Arconada, O.F.; Gabriel y Galan, J.M.; Kremer, H.P.H.; Warrenburg, B.P.C. van de

    2012-01-01

    BACKGROUND: Classically, clinical trials are based on the placebo-control design. Our aim was to analyze the placebo effect in Huntington's disease. METHODS: Placebo data were obtained from an international, longitudinal, placebo-controlled trial for Huntington's disease (European Huntington's Disea

  8. 75 FR 33617 - Notice of Proposed Settlement Agreement and Opportunity for Public Comment: West Huntington Spill...

    Science.gov (United States)

    2010-06-14

    ... AGENCY Notice of Proposed Settlement Agreement and Opportunity for Public Comment: West Huntington Spill... United States Department of Justice on behalf of EPA, in connection with the West Huntington Spill Site, Huntington, West Virginia (``Site''). DATES: Written comments on the proposed settlement agreement must...

  9. Placebo effect characteristics observed in a single, international, longitudinal study in Huntington's disease.

    NARCIS (Netherlands)

    Cubo, E.; Gonzalez, M.; Puerto, I. del; Yebenes, J.G. de; Arconada, O.F.; Gabriel y Galan, J.M.; Kremer, H.P.H.; Warrenburg, B.P.C. van de

    2012-01-01

    BACKGROUND: Classically, clinical trials are based on the placebo-control design. Our aim was to analyze the placebo effect in Huntington's disease. METHODS: Placebo data were obtained from an international, longitudinal, placebo-controlled trial for Huntington's disease (European Huntington's

  10. Disease stage, but not sex, predicts depression and psychological distress in Huntington's disease

    DEFF Research Database (Denmark)

    Dale, Maria; Maltby, John; Shimozaki, Steve

    2016-01-01

    OBJECTIVE: Depression and anxiety significantly affect morbidity in Huntington's disease. Mice. models of Huntington's disease have identified sex differences in mood-like behaviours that vary across disease lifespan, but this interaction has not previously been explored in humans with Huntington...

  11. Underground Storage Tanks - UST_IDEM_IN: Underground Storage Tanks in Indiana (Indiana Department of Environmental Management, Point Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — UST_IDEM_IN is a point shapefile that contains regulated underground storage tank locations (including leaking underground storage tanks) in Indiana, provided by...

  12. Landfills - LANDFILL_BOUNDARIES_IDEM_IN: Waste Site Boundaries in Indiana (Indiana Department of Environmental Management, Polygon Shapefile)

    Data.gov (United States)

    NSGIC State | GIS Inventory — LANDFILL_BOUNDARIES_IDEM_IN.SHP is a polygon shapefile that contains boundaries for open dump sites, approved landfills, and permitted landfills in Indiana, provided...

  13. Geology, Surficial - PHYSIOGRAPHY_SR61_IN: Physiographic Regions of Indiana (Indiana Geological Survey, 1:500,000, Polygon Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — PHYSIOGRAPHY_SR61_IN is a polygon shapefile that shows the physiographic regions of Indiana. Detailed descriptions of these regions can be obtained from Gray, H.H.,...

  14. Hydrogeology - SINKHOLE_INVENTORY_IN_KY_2011: Sinkhole inventory for southern Indiana and northern Kentucky (Indiana Geological Survey, Point Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — This point shapefile was created to display in a geographic information system (GIS) mapped and modeled (indirectly mapped) sinkhole locations in southern Indiana...

  15. Fuel Processing Plants - ETHANOL_PRODUCTION_FACILITIES_IN: Ethanol Production Facilities in Indiana (Indiana Geological Survey, Point Shapefile)

    Data.gov (United States)

    NSGIC State | GIS Inventory — This GIS layer shows the locations of ethanol production facilities in the state of Indiana. Attributes include the name and address of the facility, and information...

  16. Water Well Locations - WATERWELLS_IDNR_IN: Water-Well Locations in Indiana (Indiana Department of Natural Resources, Point Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — NOTE: The original data set named WATERWELLS_DNR_WATER_IN_100217.SHP was provided to Indiana Geological Survey personnel on February 17, 2010, personnel from the...

  17. Earthquake Risk - EARTHQUAKE_LIQUEFACTION_IN: Earthquake Paleoliquefaction Sites in Indiana (Indiana Geological Survey, 1:24,000, Point Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — EARTHQUAKE_LIQUEFACTION_IN is a point shapefile that shows sites where paleoliquefaction features have been identified in the field by Pat Munson of the Indiana...

  18. Fuel Processing Plants - ETHANOL_PRODUCTION_FACILITIES_IN: Ethanol Production Facilities in Indiana (Indiana Geological Survey, Point Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — This GIS layer shows the locations of ethanol production facilities in the state of Indiana. Attributes include the name and address of the facility, and information...

  19. Aggregate and Mineral Resources - INDUSTRIAL_MINERALS_POINTS_IN: Industrial Mineral Data in Indiana (Indiana Geological Survey, Point Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — INDUSTRIAL_MINERALS_POINTS_IN is a shapefile that shows the distribution of stratigraphic data for various industrial minerals in Indiana. These data were derived...

  20. Hydrogeology - HYDROGEOL_SETTINGS_IN: Hydrogeologic Terrains and Settings of Indiana (Indiana Geological Survey, 1:100,000, Polygon Shapefile)

    Data.gov (United States)

    NSGIC State | GIS Inventory — HYDROGEOL_SETTINGS_IN is a polygon shapefile that shows hydrogeologic terrains and settings of Indiana. The methodology of the investigation and definitions of terms...

  1. Huntington's disease: from molecular pathogenesis to clinical treatment.

    Science.gov (United States)

    Ross, Christopher A; Tabrizi, Sarah J

    2011-01-01

    Huntington's disease is a progressive, fatal, neurodegenerative disorder caused by an expanded CAG repeat in the huntingtin gene, which encodes an abnormally long polyglutamine repeat in the huntingtin protein. Huntington's disease has served as a model for the study of other more common neurodegenerative disorders, such as Alzheimer's disease and Parkinson's disease. These disorders all share features including: delayed onset; selective neuronal vulnerability, despite widespread expression of disease-related proteins during the whole lifetime; abnormal protein processing and aggregation; and cellular toxic effects involving both cell autonomous and cell-cell interaction mechanisms. Pathogenic pathways of Huntington's disease are beginning to be unravelled, offering targets for treatments. Additionally, predictive genetic testing and findings of neuroimaging studies show that, as in some other neurodegenerative disorders, neurodegeneration in affected individuals begins many years before onset of diagnosable signs and symptoms of Huntington's disease, and it is accompanied by subtle cognitive, motor, and psychiatric changes (so-called prodromal disease). Thus, Huntington's disease is also emerging as a model for strategies to develop therapeutic interventions, not only to slow progression of manifest disease but also to delay, or ideally prevent, its onset.

  2. Investigational agents for the management of Huntington's disease.

    Science.gov (United States)

    Müller, Thomas

    2017-02-01

    An inherited, chronic progressive, neurodegenerative disorder is Huntington's disease, characterized by motor, cognitive, and psychiatric symptoms. Predictive genetic testing allows earlier diagnosis and identification of gene carriers for Huntington's disease. These individuals are ideal candidates for testing of therapeutic interventions for disease modification. Areas covered: According to queries in Pubmed, Embase and clinical register databases, research and clinical studies emerge on symptomatic and neuroprotective therapies in Huntington's disease. This review discusses novel agents for symptomatic therapy and disease modification. They are currently in phase I and II of drug development Expert opinion: There are promising, safe and well tolerated compounds for amelioration of motor and neuropsychiatric symptoms, but their efficacy still needs to be proven in clinical trials. Deterioration of mutant huntingtin expression, antiapoptotic or cell death inhibition as disease modifying concepts was efficacious in models of Huntington's disease. However, the risk for clinical trial failures is high not only due to ineffectiveness of the tested agent. Negative study outcomes may also result from design misconceptions, underestimation of the heterogeneity of Huntington's disease, too short study durations and too small study cohorts.

  3. Single sperm analysis of the trinucleotide repeat in the Huntington`s disease gene

    Energy Technology Data Exchange (ETDEWEB)

    Leeflang, E.P.; Zhang, L.; Hubert, R. [Univ. of Southern California, Los Angeles, CA (United States)] [and others

    1994-09-01

    Huntington`s disease (HD) is one of several genetic diseases caused by trinucleotide repeat expansion. The CAG repeat is very unstable, with size changes occurring in more than 80% of transmissions. The degree of instability of this repeat in the male germline can be determined by analysis of individual sperm cells. An easy and sensitive PCR assay has been developed to amplify this trinucleotide repeat region from single sperm using two rounds of PCR. As many as 90% of the single sperm show amplification for the HD repeat. The PCR product can be easily detected on an ethidium bromide-stained agarose gel. Single sperm samples from an HD patient with 18 and 49 repeats were studied. We observed size variations for the expanded alleles while the size of the normal allele in sperm is very consistent. We did not detect any significant bias in the amplification of normal alleles over the larger HD alleles. Our preliminary study supports the observation made by PCR of total sperm that instability of the HD trinucleotide repeat occurs in the germline. HD preimplantation diagnosis on single embryo blastomeres may also possible.

  4. 22 Years of predictive testing for Huntington's disease: the experience of the UK Huntington's Prediction Consortium.

    Science.gov (United States)

    Baig, Sheharyar S; Strong, Mark; Rosser, Elisabeth; Taverner, Nicola V; Glew, Ruth; Miedzybrodzka, Zosia; Clarke, Angus; Craufurd, David; Quarrell, Oliver W

    2016-10-01

    Huntington's disease (HD) is a progressive neurodegenerative condition. At-risk individuals have accessed predictive testing via direct mutation testing since 1993. The UK Huntington's Prediction Consortium has collected anonymised data on UK predictive tests, annually, from 1993 to 2014: 9407 predictive tests were performed across 23 UK centres. Where gender was recorded, 4077 participants were male (44.3%) and 5122 were female (55.7%). The median age of participants was 37 years. The most common reason for predictive testing was to reduce uncertainty (70.5%). Of the 8441 predictive tests on individuals at 50% prior risk, 4629 (54.8%) were reported as mutation negative and 3790 (44.9%) were mutation positive, with 22 (0.3%) in the database being uninterpretable. Using a prevalence figure of 12.3 × 10(-5), the cumulative uptake of predictive testing in the 50% at-risk UK population from 1994 to 2014 was estimated at 17.4% (95% CI: 16.9-18.0%). We present the largest study conducted on predictive testing in HD. Our findings indicate that the vast majority of individuals at risk of HD (>80%) have not undergone predictive testing. Future therapies in HD will likely target presymptomatic individuals; therefore, identifying the at-risk population whose gene status is unknown is of significant public health value.

  5. A study on the trinucleotide repeat associated with Huntington`s disease in the Chinese

    Energy Technology Data Exchange (ETDEWEB)

    Bing-wen Soong; Jih-tsuu Wang [Neurological Institute, Taipei (Taiwan, Province of China)

    1994-09-01

    Analysis of the polymorphic (CAG)n repeat in the hungingtin gene in the chinese confirmed the presence of an expanded repeat on all Huntington`s disease chromosomes. Measurement of the specific CAG repeat sequence in 34 HD chromosomes from 15 unrelated families and 190 control chromosomes from the Chinese population showed a range from 9 to 29 repeats in normal subjects and 40 to 58 in affected subjects. The size distributions of normal and affected alleles did not overlap. A clear correlation bewteen early onset of symptoms and very high repeat number was seen, but the spread of the age-at-onset in the major repeat range producing characteristic HD it too wide to be of diagnostic value. There was also variability in the transmitted repeat size for both sexes in the HD size range. Maternal HD alleles showed a moderate instability with a preponderance of size decrease, while paternal HD alleles had a tendency to increase in repeat size on transmission, the degree of which appeared proportional to the initial size.

  6. The Frequency of Huntington Disease and Huntington Disease-Like 2 in the South African Population.

    Science.gov (United States)

    Baine, Fiona K; Krause, Amanda; Greenberg, L Jacquie

    2016-01-01

    Huntington disease (HD) has most recently been estimated to affect between 10.6 and 13.7 per 100,000 individuals in European populations. However, prevalence is known to differ geographically. In South Africa, the only published estimates are from a survey performed in the 1970s, an era when the disease was believed to be rare or absent in black individuals and molecular confirmation was absent. The disease phenotype in South Africa is currently attributable to mutations in both the huntington and junctophilin-3 genes, which underlie the well-known HD and the rarer HD-like 2 (HDL2) respectively. This study aimed at providing improved minimum estimates of disease frequency in South Africa, based on molecular genetic testing data. A review of all testing records for HD and HDL2 over a 20-year period was undertaken. HDL2 is virtually indistinguishable on clinical features, thus necessitating its inclusion. Based on molecular diagnostic records, minimum estimates of disease frequency are: 5.1, 2.1 and 0.25 (per 100,000 individuals) for the white, mixed ancestry and black population groups respectively. Although ascertainment remains incomplete, these minimum estimates suggest that disease frequencies are significantly higher than those previously reported in South Africa. © 2016 S. Karger AG, Basel.

  7. Association of Huntington's disease and schizophrenia-like psychosis in a Huntington's disease pedigree

    Directory of Open Access Journals (Sweden)

    Guimarães João

    2006-02-01

    Full Text Available Abstract Background Huntington's disease (HD is a dominantly inherited, neurodegenerative disorder due to expansion of a polymorphic trinucleotide repeat in the short arm of chromosome 4. Clinical manifestations consist of a triad of choreic movements, cognitive decline and psychiatric syndromes starting in the fourth to fifth decade. Psychiatric manifestations vary and may precede motor and cognitive changes. Personality changes and depression occur most commonly. Paranoid schizophrenia-like symptoms occur in 6% to 25% of cases. Case report We describe a 55 year-old woman with an 8 yearlong history of behavioural changes, multi-thematic delusions and auditory hallucinations. History and mental state examination were suggestive of paranoid schizophrenia. Neurological examination revealed discrete, involuntary movements affecting her arms and trunk. Genotyping detected an expanded allele (43 trinucleotide repeats. A three-generation-long family history of chorea and schizophrenia-like psychosis was found. Conclusion HD-families have been reported in which schizophrenia-like syndromes emerged in all or most HD-affected members long before they developed extra-pyramidal or cognitive changes. This has been attributed to more than mere coincidence. We hypothesise that in these families the HD gene is transmitted along with a low load of small-effect "psychosis genes" which, in the presence of the severe cognitive changes of HD, manifest as a schizophrenia-like phenotype. Further research is needed in order to clarify the links between genetic loading and the emergence of psychotic symptoms in Huntington's disease.

  8. Exclusion testing in pregnancy for Huntington's disease.

    Science.gov (United States)

    Tyler, A; Quarrell, O W; Lazarou, L P; Meredith, A L; Harper, P S

    1990-01-01

    The results of DNA analysis are presented for a series of 90 couples, with one partner at 50% risk for Huntington's disease (HD), who were referred for exclusion testing in pregnancy over a three year period. Thirty-seven couples were studied in detail. The aims of the study were to evaluate attitudes towards prenatal testing, before pregnancy and afterwards, and the effectiveness of our counseling and methods of organising the service. Problems which could arise in relation to presymptomatic testing are documented. It is concluded that exclusion testing is a valuable form of prediction for some couples, particularly where family structure does not permit prediction for the person at risk. The need for intensive counselling was highlighted by the difficulties experienced by many couples in understanding how the test worked. Particular ethical and organisational problems may arise which require careful consideration beforehand and some recommendations are made. The proportion of couples who will continue to request exclusion testing as pre-symptomatic testing becomes more widely applicable remains unknown. PMID:2145437

  9. Cell-based technologies for Huntington's disease

    Directory of Open Access Journals (Sweden)

    Mônica Santoro Haddad

    Full Text Available ABSTRACT Huntington's disease (HD is a fatal genetic disorder, which causes the progressive breakdown of neurons in the human brain. HD deteriorates human physical and mental abilities over time and has no cure. Stem cell-based technologies are promising novel treatments, and in HD, they aim to replace lost neurons and/or to prevent neural cell death. Herein we discuss the use of human fetal tissue (hFT, neural stem cells (NSCs of hFT origin or embryonic stem cells (ESCs and induced pluripotent stem cells (IPSCs, in clinical and pre-clinical studies. The in vivo use of mesenchymal stem cells (MSCs, which are derived from non-neural tissues, will also be discussed. All these studies prove the potential of stem cells for transplantation therapy in HD, demonstrating cell grafting and the ability to differentiate into mature neurons, resulting in behavioral improvements. We claim that there are still many problems to overcome before these technologies become available for HD patient treatment, such as: a safety regarding the use of NSCs and pluripotent stem cells, which are potentially teratogenic; b safety regarding the transplantation procedure itself, which represents a risk and needs to be better studied; and finally c technical and ethical issues regarding cells of fetal and embryonic origin.

  10. Comprehension of prosody in Huntington's disease.

    Science.gov (United States)

    Speedie, L J; Brake, N; Folstein, S E; Bowers, D; Heilman, K M

    1990-07-01

    Patients with Huntington's Disease (HD) who were without dementia were compared to unilateral stroke patients and controls as previously reported in 1983, to discover if they had a prosodic defect. Subjects were presented tape-recorded speech filtered sentences and asked to indicate the tone of voice as happy, sad or angry (affective prosody), or as a question, command or statement (propositional prosody). HD patients were impaired in comprehension of both types of prosody compared to controls but were not different from stroke patients. A second study compared early HD patients with at-risk siblings and spouse controls on comprehension of affective and propositional prosody, discrimination of both types of prosody, rhythm discrimination and tonal memory (Seashore tests). HD patients were impaired in both comprehension and discrimination of all types of prosody. HD patients were less accurate than at-risk patients on the tonal memory task but not on the rhythm discrimination task. These findings suggest compromise in ability to understand the more subtle prosodic aspects of communication which may contribute to social impairment of HD patients very early in the course of the disease.

  11. Hypothalamic-endocrine aspects in Huntington's disease.

    Science.gov (United States)

    Petersén, Asa; Björkqvist, Maria

    2006-08-01

    Huntington's disease (HD) is a hereditary and fatal disorder caused by an expanded CAG triplet repeat in the HD gene, resulting in a mutant form of the protein huntingtin. Wild-type and mutant huntingtin are expressed in most tissues of the body but the normal function of huntingtin is not fully known. In HD, the neuropathology is characterized by intranuclear and cytoplasmic inclusions of huntingtin aggregates, and cell death primarily in striatum and cerebral cortex. However, hypothalamic atrophy occurs at early stages of HD with loss of orexin- and somatostatin-containing cell populations. Several symptoms of HD such as sleep disturbances, alterations in circadian rhythm, and weight loss may be due to hypothalamic dysfunction. Endocrine changes including increased cortisol levels, reduced testosterone levels and increased prevalence of diabetes are found in HD patients. In HD mice, alterations in the hypothalamic-pituitary-adrenal axis occurs as well as pancreatic beta-cell and adipocyte dysfunction. Increasing evidence points towards important pathology of the hypothalamus and the endocrine system in HD. As many neuroendocrine factors are secreted into the cerebrospinal fluid, blood and urine, it is possible that their levels may reflect the disease state in the central nervous system. Investigating neuroendocrine changes in HD opens up the possibility of finding biomarkers to evaluate future therapies for HD, as well as of identifying novel targets for therapeutic interventions.

  12. DNA instability in replicating Huntington's disease lymphoblasts

    Directory of Open Access Journals (Sweden)

    Frati Luigi

    2009-02-01

    Full Text Available Abstract Background The expanded CAG repeat in the Huntington's disease (HD gene may display tissue-specific variability (e.g. triplet mosaicism in repeat length, the longest mutations involving mitotic (germ and glial cells and postmitotic (neurons cells. What contributes to the triplet mutability underlying the development of HD nevertheless remains unknown. We investigated whether, besides the increased DNA instability documented in postmitotic neurons, possible environmental and genetic mechanisms, related to cell replication, may concur to determine CAG repeat mutability. To test this hypothesis we used, as a model, cultured HD patients' lymphoblasts with various CAG repeat lengths. Results Although most lymphoblastoid cell lines (88% showed little or no repeat instability even after six or more months culture, in lymphoblasts with large expansion repeats beyond 60 CAG repeats the mutation size and triplet mosaicism always increased during replication, implying that the repeat mutability for highly expanded mutations may quantitatively depend on the triplet expansion size. None of the investigated genetic factors, potentially acting in cis to the mutation, significantly influence the repeat changes. Finally, in our experiments certain drugs controlled triplet expansion in two prone-to-expand HD cell lines carrying large CAG mutations. Conclusion Our data support quantitative evidence that the inherited CAG length of expanded alleles has a major influence on somatic repeat variation. The longest triplet expansions show wide somatic variations and may offer a mechanistic model to study triplet drug-controlled instability and genetic factors influencing it.

  13. Genetic diagnosis of Huntington's disease: cases report

    Institute of Scientific and Technical Information of China (English)

    Liao Ting-ting; Wu Wei; Wan Qi; Cui Yu-gui; Liu Jia-yin

    2011-01-01

    Objective:To assess the efficiency of the PCR combined DNA sequencing to ascertain CAG repeat size of Huntington's disease(HD)gene as for gene diagnosis of HD.Method:Three patients with HD were diagnosed genetically with the technology of polymerase chain reaction (PCR) and polyacrylamide gel electrophoresis by assessing the CAG repeat size of HD gene.DNA sequencing then was used as verification test for HD gene.Results:Nine members of three nuclear families were included in this study,three patients were HD proband.In those families,CAG repeats of all spouse of propositus were in normal range.CAG repeats of all propositus and their descendants with the normal allele were in normal range,while CAG copy number of the other mobigenous allele was obviously abnormal.Conclusion:PCR combined DNA sequencing can be used to effectively ascertain CAG repeat of HD gene.CAG-repeat expansion mutations were accounted for 99% of HD cases,so HD can be accurately diagnosed by this method.

  14. Genetic Testing for Huntington's Disease in Parkinsonism.

    Science.gov (United States)

    Rahman, M S; Nagai, Y; Popiel, H A; Fujikake, N; Okamoto, Y; Ahmed, M U; Islam, M A; Islam, M T; Ahmed, S; Rahman, K M; Uddin, M J; Dey, S K; Ahmed, Q; Hossain, M A; Jahan, N; Toda, T

    2010-10-01

    The study was conducted to find out Huntington's disease (HD) by genetic analysis from those presenting with parkinsonism in the Neurology department of Mymensingh Medical College & Hospital. A sample of about 5ml blood was collected by veni puncture in EDTA tube with informed consent from 9 patients & 7 healthy individuals after approval of the institutional ethics committee for genetic study. The neurological disorder along with a complete history and physical findings were recorded in a prescribed questionnaire by the neurologists of Mymensingh Medical College & Hospital. Extraction of genomic DNA from the venous blood using FlexiGene DNA kit (Qiagen, Japan) was performed in Faculty of Veterinary Science, Bangladesh Agricultural University, Mymensingh, Bangladesh. The extracted DNA was stored and accumulated and then these DNA were sent to Division of Clinical Genetics, Department of Medical Genetics, Osaka University Medical School, Suita, Osaka 565 0871, Japan for PCR and further analysis. PCR amplification of the CAG repeat in the 1T15 gene was performed with primers HD1 and HD3. HD PCR products revealed the DNA product of about 110bp (no. of CAG repeats=21) to 150bp (no. of CAG repeats=34) in both healthy individual and suspected PD patient DNA.

  15. Pridopidine for the treatment of Huntington's disease.

    Science.gov (United States)

    Shannon, Kathleen M

    2016-01-01

    Huntington's disease is a rare dominantly-inherited neurodegenerative disease with motor, cognitive and behavioral manifestations. It results from an expanded unstable trinucleotide repeat in the coding region of the huntingtin gene. Treatment is symptomatic, but a poor evidence baseguides selection of therapeutic agents. Non-choreic derangements in voluntary movement contribute to overall motor disability and are poorly addressed by current therapies. Pridopidine is a novel agent in the dopidine class believed to have 'state dependent' effects at dopamine receptors, thus show promise in the treatment of these disorders of voluntary movement. This review discusses the pharmacokinetics and pharmacodynamics of pridopidine and reviews clinical trials supporting development of the drug for HD. This information was culled from literature searches for dopidines, pridopidine, and HD experimental therapeutics in PubMed and at http://www.clinicaltrials.org . There is a compelling need to discover new treatments for motor disability in HD, particularly for non-choreic motor symptoms. While pridopidine failed to achieve its primary efficacy outcomes in 2 large trials, reproducible effects on secondary motor outcomes have fueled an ongoing trial studying higher doses and more focused clinical endpoints. This and phase III trials will define define the utility of pridopidine for HD.

  16. Lessons Learned from the Transgenic Huntington's Disease Rats

    Directory of Open Access Journals (Sweden)

    Rinske Vlamings

    2012-01-01

    Full Text Available Huntington's disease (HD is a fatal inherited disorder leading to selective neurodegeneration and neuropsychiatric symptoms. Currently, there is no treatment to slow down or to stop the disease. There is also no therapy to effectively reduce the symptoms. In the investigation of novel therapies, different animal models of Huntington's disease, varying from insects to nonhuman primates, have been created and used. Few years ago, the first transgenic rat model of HD, carrying a truncated huntingtin cDNA fragment with 51 CAG repeats under control of the native rat huntingtin promoter, was introduced. We have been using this animal model in our research and review here our experience with the behavioural, neurophysiological, and histopathological phenotype of the transgenic Huntington's disease rats with relevant literature.

  17. Variation within the Huntington's disease gene influences normal brain structure.

    Directory of Open Access Journals (Sweden)

    Mark Mühlau

    Full Text Available Genetics of the variability of normal and diseased brain structure largely remains to be elucidated. Expansions of certain trinucleotide repeats cause neurodegenerative disorders of which Huntington's disease constitutes the most common example. Here, we test the hypothesis that variation within the IT15 gene on chromosome 4, whose expansion causes Huntington's disease, influences normal human brain structure. In 278 normal subjects, we determined CAG repeat length within the IT15 gene on chromosome 4 and analyzed high-resolution T1-weighted magnetic resonance images by the use of voxel-based morphometry. We found an increase of GM with increasing long CAG repeat and its interaction with age within the pallidum, which is involved in Huntington's disease. Our study demonstrates that a certain trinucleotide repeat influences normal brain structure in humans. This result may have important implications for the understanding of both the healthy and diseased brain.

  18. 75 FR 2090 - Approval and Promulgation of Air Quality Implementation Plans; Indiana; Volatile Organic Compound...

    Science.gov (United States)

    2010-01-14

    ... Organic Compound Automobile Refinishing Rules for Indiana AGENCY: Environmental Protection Agency (EPA... relations, Nitrogen dioxide, Ozone, Reporting and recordkeeping requirements, Volatile organic compounds... Plan (SIP). These rule revisions extend the applicability of Indiana's approved volatile...

  19. Cost-Effectiveness Analysis of the Residential Provisions of the 2015 IECC for Indiana

    Energy Technology Data Exchange (ETDEWEB)

    Mendon, Vrushali V.; Zhao, Mingjie; Taylor, Zachary T.; Poehlman, Eric A.

    2016-02-15

    The 2015 IECC provides cost-effective savings for residential buildings in Indiana. Moving to the 2015 IECC from the 2009 IECC base code is cost-effective for residential buildings in all climate zones in Indiana.

  20. 78 FR 53781 - Notice of Inventory Completion: Indiana Department of Natural Resources, Division of Historic...

    Science.gov (United States)

    2013-08-30

    ... National Park Service Notice of Inventory Completion: Indiana Department of Natural Resources, Division of...: Notice. SUMMARY: The Indiana Department of Natural Resources, Division of Historic Preservation and... Natural Resources DHPA through the University of Indianapolis. If no additional requestors come...

  1. Changes In The Horse Racing Industry And Impacts On The Indiana Economy: 2010 - 2014

    National Research Council Canada - National Science Library

    Jonathan M. Furdek; Susan Conners

    2015-01-01

      A survey of the horse racing and horse breeding industry in Indiana was conducted in 2014 and the economic impact of this industry on the Indiana economy determined utilizing an input-output model...

  2. Training cardiac surgeons: the Indiana University experience.

    Science.gov (United States)

    Brown, John W

    2016-12-01

    In this article, I will outline the origin of cardiothoracic surgical (CTS) training at Indiana University (IU) and its evolution to the present. I will describe my educational background, surgical training in this specialty, and my role as an educator of CT surgeons. I will describe our faculty and the structure of the CTS residency. Finally, I will describe a newly adopted smart phone "App" called SIMPL, which allows the resident and faculty to quickly (50% of the most critical aspects of each surgical procedure, the resident's performance during the critical portion of the operation from poor to excellent, and the degree of difficulty of the operation from simple to complex. The attending surgeon and the resident data are then forwarded to the SIMPL database where the SIMPL software aggregates data for each resident and procedure producing a report at the end of the rotation of the resident's performance relative to his peers. This additional evaluation process will better ensure that our CTS residents are "practice ready" when they complete their training.

  3. Mercury and methylmercury in reservoirs in Indiana

    Science.gov (United States)

    Risch, Martin R.; Fredericksen, Amanda L.

    2015-01-01

    Mercury (Hg) is an element that occurs naturally, but evidence suggests that human activities have resulted in increased amounts being released to the atmosphere and land surface. When Hg is converted to methylmercury (MeHg) in aquatic ecosystems, MeHg accumulates and increases in the food web so that some fish contain levels which pose a health risk to humans and wildlife that consume these fish. Reservoirs unlike natural lakes, are a part of river systems that are managed for flood control. Data compiled and interpreted for six flood-control reservoirs in Indiana showed a relation between Hg transport, MeHg formation in water, and MeHg in fish that was influenced by physical, chemical, and biological differences among the reservoirs. Existing information precludes a uniform comparison of Hg and MeHg in all reservoirs in the State, but factors and conditions were identified that can indicate where and when Hg and MeHg levels in reservoirs could be highest.

  4. 75 FR 26198 - Foreign-Trade Zone 152 - Burns Harbor, Indiana, Application for Reorganization under Alternative...

    Science.gov (United States)

    2010-05-11

    ... Foreign-Trade Zones Board Foreign-Trade Zone 152 - Burns Harbor, Indiana, Application for Reorganization...-purpose zone currently consists of six sites in the Burns Harbor/Gary, Indiana area: Site 1: (533,288 sq...); Site 2: (441 acres) within the Port of Indiana/Burns International Harbor, Burns Harbor (Porter...

  5. Homeschooling in Indiana: A Closer Look. Education Policy Brief. Volume 3, Number 7, Summer 2005

    Science.gov (United States)

    Kunzman, Robert

    2005-01-01

    Homeschooling is quickly becoming a significant educational phenomenon. According to the Indiana Department of Education, the number of Indiana homeschooled children has increased by 400 percent over the past eight years, with a current enrollment total of 22,403. Furthermore, this number likely understates the true number of Indiana homeschooled…

  6. 77 FR 41914 - Approval and Promulgation of Air Quality Implementation Plans; Indiana

    Science.gov (United States)

    2012-07-17

    ... by the Indiana Department of Environmental Management (IDEM) on May 14, 2012, to revise the Indiana State Implementation Plan (SIP). The submission revises the Indiana Administrative Code (IAC) definition... 326 IAC 1-1-3, definition of ``References to Code of Federal Regulations.'' IDEM updated the reference...

  7. 76 FR 27904 - Approval and Promulgation of Air Quality Implementation Plans; Indiana

    Science.gov (United States)

    2011-05-13

    ... by the Indiana Department of Environmental Management (IDEM) on November 24, 2010, to revise the Indiana State Implementation Plan (SIP). The submission revises the Indiana Administrative Code (IAC) by amending and updating the definition of ``References to the Code of Federal Regulations,'' to refer to the...

  8. 76 FR 27973 - Approval and Promulgation of Air Quality Implementation Plans; Indiana

    Science.gov (United States)

    2011-05-13

    ... submitted by the Indiana Department of Environmental Management on November 24, 2010 to revise the Indiana State Implementation Plan (SIP). The submission revises the Indiana Administrative Code (IAC) by amending and updating the definition of ``References to the Code of Federal Regulations,'' to refer to the...

  9. Indiana Jones on tagasi ja näitab, kuidas käituda / Kristiina Davidjants

    Index Scriptorium Estoniae

    Davidjants, Kristiina, 1974-

    2008-01-01

    22 mail esilinastub Steven Spielbergi järjekordne Indiana Jones'i film, kaasstsenaristiks ja produtsendiks George Lucas ja Harrison Fordiga nimiosas "Indiana Jones ja kristallpealuu kuningriik" ("Indiana Jones and the Kingdom of the Crystal Skull"). Saaga varasemast kolmest filmist

  10. Indiana Jones on tagasi ja näitab, kuidas käituda / Kristiina Davidjants

    Index Scriptorium Estoniae

    Davidjants, Kristiina, 1974-

    2008-01-01

    22 mail esilinastub Steven Spielbergi järjekordne Indiana Jones'i film, kaasstsenaristiks ja produtsendiks George Lucas ja Harrison Fordiga nimiosas "Indiana Jones ja kristallpealuu kuningriik" ("Indiana Jones and the Kingdom of the Crystal Skull"). Saaga varasemast kolmest filmist

  11. The Northwest Indiana Center for Data and Analysis: A Case Study of Academic Library Community Engagement

    Science.gov (United States)

    Sandberg, Scott; Morris, Cele; Sutherland, Timothy

    2013-01-01

    This paper details community engagement activity of an academic library coordinated within a broader university strategic plan. The Anderson Library at Indiana University Northwest (IU-Northwest) supports a service called the Northwest Indiana Center for Data and Analysis. Created in 1996 with funding made available from the Indiana University…

  12. 78 FR 6035 - Approval and Promulgation of Air Quality Implementation Plans; Ohio and Indiana; Cincinnati...

    Science.gov (United States)

    2013-01-29

    ... AGENCY 40 CFR Part 52 Approval and Promulgation of Air Quality Implementation Plans; Ohio and Indiana; Cincinnati-Hamilton, OH; Ohio and Indiana 1997 8-Hour Ozone Maintenance Plan Revisions to Approved Motor..., 2010 and 77 FR 11394, February 27, 2012). The Ohio, Kentucky, Indiana Regional Council of...

  13. Tetrabenazine is neuroprotective in Huntington's disease mice

    Directory of Open Access Journals (Sweden)

    Tang Tie-Shan

    2010-04-01

    Full Text Available Abstract Background Huntington's disease (HD is a neurodegenerative disorder caused by a polyglutamine (polyQ expansion in Huntingtin protein (Htt. PolyQ expansion in Httexp causes selective degeneration of striatal medium spiny neurons (MSN in HD patients. A number of previous studies suggested that dopamine signaling plays an important role in HD pathogenesis. A specific inhibitor of vesicular monoamine transporter (VMAT2 tetrabenazine (TBZ has been recently approved by Food and Drug Administration for treatment of HD patients in the USA. TBZ acts by reducing dopaminergic input to the striatum. Results In previous studies we demonstrated that long-term feeding with TBZ (combined with L-Dopa alleviated the motor deficits and reduced the striatal neuronal loss in the yeast artificial chromosome transgenic mouse model of HD (YAC128 mice. To further investigate a potential beneficial effects of TBZ for HD treatment, we here repeated TBZ evaluation in YAC128 mice starting TBZ treatment at 2 months of age ("early" TBZ group and at 6 months of age ("late" TBZ group. In agreement with our previous studies, we found that both "early" and "late" TBZ treatments alleviated motor deficits and reduced striatal cell loss in YAC128 mice. In addition, we have been able to recapitulate and quantify depression-like symptoms in TBZ-treated mice, reminiscent of common side effects observed in HD patients taking TBZ. Conclusions Our results further support therapeutic value of TBZ for treatment of HD but also highlight the need to develop more specific dopamine antagonists which are less prone to side-effects.

  14. Everyday cognition in prodromal Huntington disease.

    Science.gov (United States)

    Williams, Janet K; Kim, Ji-In; Downing, Nancy; Farias, Sarah; Harrington, Deborah L; Long, Jeffrey D; Mills, James A; Paulsen, Jane S

    2015-03-01

    Assessment of daily functions affected by cognitive loss in prodromal Huntington's disease (HD) is necessary in practice and clinical trials. We evaluated baseline and longitudinal sensitivity of the Everyday Cognition (ECog) scales in prodromal HD and compared self- and companion-ratings. Everyday cognition was self-assessed by 850 participants with prodromal HD and 768 companions. We examined internal structure using confirmatory factor analysis (CFA) on baseline data. For longitudinal analysis, we stratified participants into Low, Medium, and High disease progression groups. We examined ECog scores for group differences and participant-and-companion differences using linear mixed effects regression (LMER). Comparison with the Total Functional Capacity (TFC) scale was made. CFA revealed good fit of a 5-factor model having a global factor (total score), and subfactors (subscales) of memory, language, visuospatial perception, and executive function. At study entry, participants and companions in the Medium and High groups reported significantly worsened everyday cognition as well as significant functional decline over time. Losses became more pronounced and participant and companion ratings diverged as individuals progressed. TFC showed significant functional loss over time in the High group but not in the Medium group. Disease progression is associated with reduced self- and companion-reported everyday cognition in prodromal HD participants who are less than 13 years to estimated motor onset. Our findings suggest companion ratings are more sensitive than participants' for detecting longitudinal change in daily cognitive function. ECog appears more sensitive to specific functional changes in the prodrome of HD than the TFC. PsycINFO Database Record (c) 2015 APA, all rights reserved.

  15. The Indiana laboratory system: focus on environmental laboratories.

    Science.gov (United States)

    Madlem, Jyl M; Hammes, Kara R; Matheson, Shelley R; Lovchik, Judith C

    2013-01-01

    The Indiana State Department of Health (ISDH) Laboratories are working to improve Indiana's state public health laboratory system. Environmental laboratories are key stakeholders in this system, but their needs have been largely unaddressed prior to this project. In an effort to identify and engage these laboratories, the ISDH Laboratories organized and hosted the First Annual Environmental Laboratories Meeting. The focus of this meeting was on water-testing laboratories throughout the state. Meeting objectives included issue identification, disaster recovery response, and communication efforts among system partners. Common concerns included the need for new technology and updated methods, analyst training, certification programs for analysts and sample collectors, electronic reporting, and regulation interpretation and inspection consistency. Now that these issues have been identified, they can be addressed through a combination of laboratory workgroups and collaboration with Indiana's regulatory agencies. Participants were overwhelmingly positive about the meeting's outcomes and were willing to help with future laboratory system improvement projects.

  16. Prevalence of conservation design in an agriculture-dominated landscape: the case of Northern Indiana.

    Science.gov (United States)

    Crick, Julie; Prokopy, Linda Stalker

    2009-06-01

    We examined the prevalence of residential development that occurs with consideration of the natural features of the site, known as conservation design, within county-level planning jurisdictions across Northern Indiana. Using data from telephone interviews with representatives of planning departments, jurisdictions were ranked based on reported use of conservation design. Three categories of use emerged from the data: no use, use of individual practices associated with conservation design, and integration of multiple conservation design practices. Qualitative data analysis revealed that conservation design practices were not being used widely and, when used, were often used to fulfill stormwater requirements. Statistical analysis, using data from interviews, spatial data sets, and the U.S. Census Bureau, identified several significant positive predictors of the levels of conservation design use including conversion of forest or agricultural land cover to urban uses and education levels in the jurisdiction. Many of the interviewees noted that agricultural land is perceived to meet open space needs within their counties. Given that agricultural land does not fully meet all ecosystem needs, education about the benefits of other types of open space is suggested.

  17. An improved assay for the determination of Huntington`s disease allele size

    Energy Technology Data Exchange (ETDEWEB)

    Reeves, C.; Klinger, K.; Miller, G. [Intergrated Genetics, Framingham, MA (United States)

    1994-09-01

    The hallmark of Huntington`s disease (HD) is the expansion of a polymorphic (CAG)n repeat. Several methods have been published describing PCR amplification of this region. Most of these assays require a complex PCR reaction mixture to amplify this GC-rich region. A consistent problem with trinucleotide repeat PCR amplification is the presence of a number of {open_quotes}stutter bands{close_quotes} which may be caused by primer or amplicon slippage during amplification or insufficient polymerase processivity. Most assays for HD arbitrarily select a particular band for diagnostic purposes. Without a clear choice for band selection such an arbitrary selection may result in inconsistent intra- or inter-laboratory findings. We present an improved protocol for the amplification of the HD trinucleotide repeat region. This method simplifies the PCR reaction buffer and results in a set of easily identifiable bands from which to determine allele size. HD alleles were identified by selecting bands of clearly greater signal intensity. Stutter banding was much reduced thus permitting easy identification of the most relevant PCR product. A second set of primers internal to the CCG polymorphism was used in selected samples to confirm allele size. The mechanism of action of N,N,N trimethylglycine in the PCR reaction is not clear. It may be possible that the minimal isostabilizing effect of N,N,N trimethylglycine at 2.5 M is significant enough to affect primer specificity. The use of N,N,N trimethylglycine in the PCR reaction facilitated identification of HD alleles and may be appropriate for use in other assays of this type.

  18. The Counselor and Genetic Disease: Huntington's Disease as a Model.

    Science.gov (United States)

    Wexler, Nancy S.

    This speech offers a brief description of Huntington's Disease (HD): its causes, symptoms, and incidence. It then concentrates on the psychological problems of persons one of whose parents had the disease, and the role of the counselor in helping these humans cope with their fears about contacting it themselves. A relatively detailed case study is…

  19. Age, CAG repeat length, and clinical progression in Huntington's disease.

    Science.gov (United States)

    Rosenblatt, Adam; Kumar, Brahma V; Mo, Alisa; Welsh, Claire S; Margolis, Russell L; Ross, Christopher A

    2012-02-01

    The objective of this study was to further explore the effect of CAG repeat length on the rate of clinical progression in patients with Huntington's disease. The dataset included records for 569 subjects followed prospectively at the Baltimore Huntington's Disease Center. Participants were seen for a mean of 7.1 visits, with a mean follow-up of 8.2 years. Subjects were evaluated using the Quantified Neurologic Examination and its Motor Impairment subscale, the Mini-Mental State Examination, and the Huntington's disease Activities of Daily Living Scale. By itself, CAG repeat length showed a statistically significant but small effect on the progression of all clinical measures. Contrary to our previous expectations, controlling for age of onset increased the correlation between CAG repeat length and progression of all variables by 69% to 159%. Graphical models further supported the idea that individuals with smaller triplet expansions experience a more gradual decline. CAG repeat length becomes an important determinant of clinical prognosis when accounting for age of onset. This suggests that the aging process itself influences clinical outcomes in Huntington's disease. Inconsistent results in prior studies examining CAG repeat length and progression may indeed reflect a lack of age adjustment.

  20. Biological Markers of Cognition in Prodromal Huntington's Disease: A Review

    Science.gov (United States)

    Papp, Kathryn V.; Kaplan, Richard F.; Snyder, Peter J.

    2011-01-01

    Huntington's disease (HD), an autosomal-dominant genetic disorder, has historically been viewed as a degenerative movement disorder but it also includes psychiatric symptoms and progressive cognitive decline. There has been a lack of consensus in the literature about whether or not cognitive signs can be detected in carriers before clinical…

  1. Huntington II Simulation Program-POLUT. Teacher's Guide.

    Science.gov (United States)

    Braun, L.; And Others

    This teacher's guide is written to accompany the Huntington II Simulation Program - POLUT. POLUT is a program written in BASIC which provides simulation of the interaction between water and waste. It creates a context within which the user can control specific variables which effect the quality of a water resource. The teacher's guide provides…

  2. Exploring Genetic Factors Involved in Huntington Disease Age of Onset

    DEFF Research Database (Denmark)

    Valcárcel-Ocete, Leire; Alkorta-Aranburu, Gorka; Iriondo, Mikel;

    2015-01-01

    Age of onset (AO) of Huntington disease (HD) is mainly determined by the length of the CAG repeat expansion (CAGexp) in exon 1 of the HTT gene. Additional genetic variation has been suggested to contribute to AO, although the mechanism by which it could affect AO is presently unknown. The aim of ...

  3. Clinical and genetic features of Huntington disease in Sri Lanka.

    Science.gov (United States)

    Sumathipala, Dulika S; Jayasekara, Rohan W; Dissanayake, Vajira H W

    2013-12-05

    Huntington disease was one of the first neurological hereditary diseases for which genetic testing was made possible as early as 1993. The study describes the clinical and genetic characteristics of patients with Huntington disease in Sri Lanka. Data of 35 consecutive patients tested from 2007 to 2012 at the Human Genetics Unit, Faculty of Medicine, University of Colombo was analyzed retrospectively. Clinical data and genetic diagnostic results were reviewed. Statistical analysis was performed using descriptive statistics. Thirty patients had fully penetrant (FP) CAG repeat mutations and 5 had reduced penetrant (RP) CAG repeat mutations. In the FP group mean ages of onset and diagnosis were 37.5 and 40.4 years, while in the RP group it was 63.0 and 64.8 years respectively. The age of diagnosis ranged from 15 to 72 years, with 2 patients with Juvenile onset (60 years) Huntington disease. The symptoms at diagnosis were predominantly motor (32/35 -91%). Three patients had psychiatric and behavioral disorders. The age difference between onset and genetic diagnosis showed significant delay in females compared to males (p Huntington disease in the Sri Lankan study population were similar to that previously reported in literature.

  4. PSYCHIATRIC ASPECTS OF HUNTINGTON DISEASE – CASE REPORTS

    Directory of Open Access Journals (Sweden)

    Mirela Batta

    2004-04-01

    Full Text Available Background. Huntington disease occurrs rarely, it can be encountered not only by neurologists and psychiatrists but also by other medical practitioners. Its characteristic features are involuntary movements, cognitive disorders and gradual development of dementia. Diagnosis is given on the basis of these clinical features, positive familial anamnesis, with the laboratory exclusion of other neuropsychiatric diseases and with the help of neuroimaging methods (in particular NMR. The disease can be only confirmed by means of genetic analysis.Patients and methods. In this article, four cases of patients with Huntington disease and diverse psychiatric disorders that were hospitalised at the psychiatric department of the Maribor General Hospital between October 2002 and March 2003 are described. All the patients fulfilled the valid criteria for the diagnosis of Huntington disease. However, they differed according to their accompanying psychiatric psychopathology, age and social problems.Conclusions. The purpose of this article is to draw attention to different psychiatric symptoms and clinical manifestations of Huntington disease that are often misleading in the diagnostic process. In addition, exigency of early diagnostics, guidelines for referrals to genetic testing and psychiatric monitoring of these patients are emphasised.

  5. Expression pattern of apoptosis-related markers in Huntington's disease

    NARCIS (Netherlands)

    Vis, José C; Schipper, Ellis; de Boer-van Huizen, Roelie T; Verbeek, Marcel M; de Waal, Rob M W; Wesseling, Pieter; ten Donkelaar, Hans J; Kremer, Berry

    2005-01-01

    Inappropriate apoptosis has been implicated in the mechanism of neuronal death in Huntington's disease (HD). In this study, we report the expression of apoptotic markers in HD caudate nucleus (grades 1-4) and compare this with controls without neurological disease. Terminal transferase-mediated biot

  6. Preparing Successful Science Teachers at Indiana University Southwest

    Directory of Open Access Journals (Sweden)

    J.E. Hollenbeck

    2008-06-01

    Full Text Available The science education program at Indiana University Southeast (IUS is successful with the adoption of a constructivist based philosophy and the National Science Education Standards (NSES. Teachers graduating from the science education program of Indiana University Southeast are qualified to be ranked as “highly qualified educators” as defined by the ‘No Child Left Behind’ Act. With the breadth and depth of knowledge and pre-service teaching experience and classroom work, IUS students are better prepared for teaching. Teacher graduates from the IUS science education program report a high degree of professional success after graduation.

  7. Employing Real Time PCR for the Diagnosis of Huntington Disease

    Directory of Open Access Journals (Sweden)

    Frouzandeh Mahjoubi

    2013-07-01

    Full Text Available Background: Huntington disease (HD is a dominantly inherited, neurodegenerative disease characterized by choreiform movement disturbances and dementia. The onset age of this disease is varied but usually is between the ages 40-50. Huntington's disease is caused by a triplet-repeat expansion in the IT15 gene (also known as huntingtin or HD which is located on chromosome 4p3.1. Since many clinical picture of HD are indistinguishable from other distinct genetic disorders molecular test such as PCR is the only way to confirm the disease. The aim of this study was to introduce a new and fast technique for the diagnosis of Huntington disease.Materials and Methods: Blood specimens were collected from individuals suspected for Huntington disease and also people with no symptoms and family history of this disease. DNAs were extracted according to standard protocol. Using conventional PCR, patient positive for Huntington disease were diagnosed. Then employing real time PCR on the basis of difference between melting temperature (Tm a new and fast diagnostic method was introduced.Results: Among 29 patients suspected to be HD only 8 HD patients were confirmed using PCR and real time PCR. The numbers of CAG repeat were between 42-50 and melting temperatures were between 89-92.Conclusion: The concept of using melting temperature in real time PCR protocol presented in here could be employed for the rapid diagnosis of the diseases caused by the increased in triple repeat sequences. It is fast, robust and has the potential use for the prenatal diagnosis.

  8. Major Superficial White Matter Abnormalities in Huntington's Disease

    Science.gov (United States)

    Phillips, Owen R.; Joshi, Shantanu H.; Squitieri, Ferdinando; Sanchez-Castaneda, Cristina; Narr, Katherine; Shattuck, David W.; Caltagirone, Carlo; Sabatini, Umberto; Di Paola, Margherita

    2016-01-01

    Background: The late myelinating superficial white matter at the juncture of the cortical gray and white matter comprising the intracortical myelin and short-range association fibers has not received attention in Huntington's disease. It is an area of the brain that is late myelinating and is sensitive to both normal aging and neurodegenerative disease effects. Therefore, it may be sensitive to Huntington's disease processes. Methods: Structural MRI data from 25 Pre-symptomatic subjects, 24 Huntington's disease patients and 49 healthy controls was run through a cortical pattern-matching program. The surface corresponding to the white matter directly below the cortical gray matter was then extracted. Individual subject's Diffusion Tensor Imaging (DTI) data was aligned to their structural MRI data. Diffusivity values along the white matter surface were then sampled at each vertex point. DTI measures with high spatial resolution across the superficial white matter surface were then analyzed with the General Linear Model to test for the effects of disease. Results: There was an overall increase in the axial and radial diffusivity across much of the superficial white matter (p < 0.001) in Pre-symptomatic subjects compared to controls. In Huntington's disease patients increased diffusivity covered essentially the whole brain (p < 0.001). Changes are correlated with genotype (CAG repeat number) and disease burden (p < 0.001). Conclusions: This study showed broad abnormalities in superficial white matter even before symptoms are present in Huntington's disease. Since, the superficial white matter has a unique microstructure and function these abnormalities suggest it plays an important role in the disease. PMID:27242403

  9. Flood-inundation maps for the Mississinewa River at Marion, Indiana, 2013

    Science.gov (United States)

    Coon, William F.

    2014-01-01

    Digital flood-inundation maps for a 9-mile (mi) reach of the Mississinewa River from 0.75 mi upstream from the Pennsylvania Street bridge in Marion, Indiana, to 0.2 mi downstream from State Route 15 were created by the U.S. Geological Survey (USGS) in cooperation with the Indiana Office of Community and Rural Affairs. The flood inundation maps, which can be accessed through the USGS Flood Inundation Mapping Science Web site at http://water.usgs.gov/osw/flood_inundation, depict estimates of the areal extent and depth of flooding corresponding to selected water levels (stages) at the USGS streamgage on the Mississinewa River at Marion (station number 03326500). Near-real-time stages at this streamgage may be obtained on the Internet from the USGS National Water Information System at http://waterdata.usgs.gov/ or the National Weather Service (NWS) Advanced Hydrologic Prediction Service at http://water.weather.gov/ahps/, which also forecasts flood hydrographs at this site. Flood profiles were computed for the stream reach by means of a one-dimensional step-backwater model. The model was calibrated by using the current stage-discharge relation at the Mississinewa River streamgage, in combination with water-surface profiles from historic floods and from the current (2002) flood-insurance study for Grant County, Indiana. The hydraulic model was then used to compute seven water-surface profiles for flood stages at 1-fo (ft) intervals referenced to the streamgage datum and ranging from 10 ft, which is near bankfull, to 16 ft, which is between the water levels associated with the estimated 10- and 2-percent annual exceedance probability floods (floods with recurrence interval between 10 and 50 years) and equals the “major flood stage” as defined by the NWS. The simulated water-surface profiles were then combined with a Geographic Information System digital elevation model (derived from light detection and ranging (lidar) data having a 0.98 ft vertical accuracy and 4.9 ft

  10. Flood-inundation maps for the Tippecanoe River near Delphi, Indiana

    Science.gov (United States)

    Menke, Chad D.; Bunch, Aubrey R.; Kim, Moon H.

    2013-01-01

    Digital flood-inundation maps for an 11-mile reach of the Tippecanoe River that extends from County Road W725N to State Road 18 below Oakdale Dam, Indiana (Ind.), were created by the U.S. Geological Survey (USGS) in cooperation with the Indiana Department of Transportation. The inundation maps, which can be accessed through the USGS Flood Inundation Mapping Science Web site at http://water.usgs.gov/osw/flood_inundation/, depict estimates of the areal extent of flooding corresponding to selected water levels (stages) at USGS streamgage 03333050, Tippecanoe River near Delphi, Ind. Current conditions at the USGS streamgages in Indiana may be obtained online at http://waterdata.usgs.gov/in/nwis/current/?type=flow. In addition, the information has been provided to the National Weather Service (NWS) for incorporation into their Advanced Hydrologic Prediction Service (AHPS) flood warning system (http://water.weather.gov/ahps/). The NWS forecasts flood hydrographs at many places that are often co-located at USGS streamgages. That forecasted peak-stage information, also available on the Internet, may be used in conjunction with the maps developed in this study to show predicted areas of flood inundation. In this study, water-surface profiles were simulated for the stream reach by means of a hydraulic one-dimensional step-backwater model. The model was calibrated by using the most current stage-discharge relation at USGS streamgage 03333050, Tippecanoe River near Delphi, Ind., and USGS streamgage 03332605, Tippecanoe River below Oakdale Dam, Ind. The hydraulic model was then used to simulate 13 water-surface profiles for flood stages at 1-foot intervals reference to the streamgage datum and ranging from bankfull to approximately the highest recorded water level at the streamgage. The simulated water-surface profiles were then combined with a geographic information system digital elevation model (derived from Light Detection and Ranging (LiDAR) data) in order to delineate the

  11. Chemical and biological quality of streams at the Indiana Dunes National Lakeshore, Indiana, 1978-80

    Science.gov (United States)

    Hardy, M.A.

    1984-01-01

    A variety of land uses affects water quality of streams at the Indiana Dunes National Lakeshore. Discharge from storm sewers and runoff from roads contributed lead, zinc, and chlorinated hydrocarbons (chlordane, DOT, ODD, DDE, and PCB's) to all streams except Derby ditch. In addition, the Little Calumet River received ammonia from industrial discharges, and organic materials, nitrogen, phosphorus, and fecal coliform from wastewater-treatment-plant and combinedsanitary- and storm-sewer discharges. As a result, water at some sites in the lower reaches of the Little Calumet River contained dissolved-ammonium-nitrogen concentrations exceeding 0.10 milligram per liter, dissolved-oxygen concentrations less than 3.0 milligrams per liter, and fecal coliform populations exceeding 2,000 colonies per 100 milliliters.

  12. Targeting the Cholinergic System to Develop a Novel Therapy for Huntington's Disease.

    Science.gov (United States)

    D'Souza, Gary X; Waldvogel, Henry J

    2016-12-15

    In this review, we outline the role of the cholinergic system in Huntington's disease, and briefly describe the dysfunction of cholinergic transmission, cholinergic neurons, cholinergic receptors and cholinergic survival factors observed in post-mortem human brains and animal models of Huntington's disease. We postulate how the dysfunctional cholinergic system can be targeted to develop novel therapies for Huntington's disease, and discuss the beneficial effects of cholinergic therapies in pre-clinical and clinical studies.

  13. Crime in Huntington's disease: a study of registered offences among patients, relatives, and controls

    OpenAIRE

    Jensen, P; Fenger, K; Bolwig, T; Sorensen, S. A.

    1998-01-01

    OBJECTIVES—Criminal behaviour has been described as a problem in Huntington's disease, but systematic studies including control groups have been missing. Based on information from Danish registries, rates and types of crime committed by patients with Huntington's disease, non-affected relatives, and controls were studied.
METHODS—99 males and 151 females with Huntington's disease were compared with 334 non-affected first degree relatives (134 men and 200 women) and to matche...

  14. Special Education Hearings in Indiana: An Analysis of Fiscal Issues.

    Science.gov (United States)

    Wood, R. Craig; Carros, Dawn

    1988-01-01

    Investigates due process hearing officer decisions in issues (under the Education for All Handicapped Children Act) that could result in increased local education agency (LEA) expenditures in Indiana. Parents prevailed in 64 percent of the cases, indicating no hearing officer bias against increased LEA expenditures for required additional…

  15. Studying Indiana Public Libraries' Usage of Internet Filters

    Science.gov (United States)

    Comer, Alberta Davis

    2005-01-01

    A lot of literature from the past few years has discussed the importance of bridging the digital divide that exists between those with Internet access and those without. Public libraries have often declared themselves to be that bridge. This article looks at how Indiana's public libraries allow the citizens they serve access to the Internet. With…

  16. Indiana AIDS Prevention Plan, 1986. Version 1.0.

    Science.gov (United States)

    Indiana State Board of Health, Indianapolis.

    The Indiana statewide Acquired Immune Deficiency Syndrome (AIDS) prevention plan focuses on community education efforts targeted for specific high risk groups as well as health care and other professionals. Plans are summarized for dissemination of information to the following groups: risk groups, physicians, dental health, nursing, ancillary…

  17. 2003 Public Opinion Survey on Education in Indiana

    Science.gov (United States)

    Plucker, Jonathan A.; Chamberlin, Molly A.; Zapf, Jason S.; Simmons, Ada B.

    2004-01-01

    The purposes of the 2003 Public Opinion Survey on Education in Indiana are to serve as a standardized approach for reliable measurement of public opinion, to allow for reliable comparisons to results of similar survey research projects, and to provide a research methodology that facilitates replication in the future and comparisons of results…

  18. 2006 Public Opinion Survey on Education in Indiana

    Science.gov (United States)

    Plucker, Jonathan A.; Spradlin, Terry E.; Zapf, Jason S.; Chien, Rosanne W.

    2007-01-01

    The 2006 Public Opinion Survey on Education in Indiana gauged the attitudes and perceptions of a representative sample of Hoosiers on such key educational issues as kindergarten and pre-kindergarten programs, No Child Left Behind and P.L. 221, school funding and taxes, teacher quality, school choice and charter schools, and the achievement gap in…

  19. Using GIS to analyse dental practice distribution in Indiana, USA.

    Science.gov (United States)

    Kurcz, R; Kruger, E; Tennant, M

    2013-09-01

    Dentistry across the globe faces significant workforce issues with mal-distribution at most levels of analysis being a substantial issue. This study was the first to apply high resolution Geographic Information Systems (GIS) tools to map every private dental practice in the State of Indiana against a backdrop of population demographics. The hypothesis tested in the study is that there is an even density distribution of dental practices across Indiana. Adult population data were obtained from the United States Census of Population and divided by census tracts. The physical address for each dental practice in Indiana was collated from a comprehensive web-based search and the two datasets were integrated using GIS tools. The whole adult population of Indiana (5 million) was distributed across 1,511 census tracts. Across these tracts a total of 2,096 separate private general dental practices were distributed. There were a total of 679 tracts (45%) without a dental practice while 2.5% of tracts had 8 or more practices. The practice to population ratio (1:2,384) for the whole State was not significantly different for those living within 50km (31 miles) or 25km (15 miles) of the seven major city centers, and mean personal income (by residency location) did not appear to significantly influence practice location.

  20. Cultural Resources of the Ohio River Valley in Indiana,

    Science.gov (United States)

    1977-07-01

    1963) "Early Crab Orchard" Sugar Hill cordmarked pottery (Maxwell 1951:273-274) Crab Orchard series pottery without nodes (MIaxwell 1951:274- 278...Fuller. Madison. Brunett , F. n.d. Plant remains from the Angel Mounds site, Indiana. Masters Thesis (unpublished), University of Michigan, Ann Arbor

  1. Caracterizacion Lexica del Espanol Hablado en el Noroeste de Indiana (Lexical Characterization of the Spanish Spoken in Northwest Indiana).

    Science.gov (United States)

    Mendieta, Eva; Molina, Isabel

    2000-01-01

    Analyzes Spanish lexical data recorded in sociolinguistic interviews with Hispanic community members in Northwest Indiana. Examined how prevalent English is in the spoken Spanish of this community; what variety of Spanish is regarded prestigious; whether lexical forms establish the prestige dialect adopted by speakers of other dialects; the…

  2. Caracterizacion Lexica del Espanol Hablado en el Noroeste de Indiana (Lexical Characterization of the Spanish Spoken in Northwest Indiana).

    Science.gov (United States)

    Mendieta, Eva; Molina, Isabel

    2000-01-01

    Analyzes Spanish lexical data recorded in sociolinguistic interviews with Hispanic community members in Northwest Indiana. Examined how prevalent English is in the spoken Spanish of this community; what variety of Spanish is regarded prestigious; whether lexical forms establish the prestige dialect adopted by speakers of other dialects; the…

  3. Family caregivers' views on coordination of care in Huntington's disease

    DEFF Research Database (Denmark)

    Røthing, Merete; Malterud, Kirsti; Frich, Jan C

    2015-01-01

    BACKGROUND: Collaboration between family caregivers and health professionals in specialised hospitals or community-based primary healthcare systems can be challenging. During the course of severe chronic disease, several health professionals might be involved at a given time, and the patient......'s illness may be unpredictable or not well understood by some of those involved in the treatment and care. AIM: The aim of this study was to explore the experiences and expectations of family caregivers for persons with Huntington's disease concerning collaboration with healthcare professionals. METHODS......: To shed light on collaboration from the perspectives of family caregivers, we conducted an explorative, qualitative interview study with 15 adult participants experienced from caring for family members in all stages of Huntington's disease. Data were analysed with systematic text condensation, a cross...

  4. Psychodynamic theory and counseling in predictive testing for Huntington's disease.

    Science.gov (United States)

    Tassicker, Roslyn J

    2005-04-01

    This paper revisits psychodynamic theory, which can be applied in predictive testing counseling for Huntington's Disease (HD). Psychodynamic theory has developed from the work of Freud and places importance on early parent-child experiences. The nature of these relationships, or attachments are reflected in adult expectations and relationships. Two significant concepts, identification and fear of abandonment, have been developed and expounded by the psychodynamic theorist, Melanie Klein. The processes of identification and fear of abandonment can become evident in predictive testing counseling and are colored by the client's experience of growing up with a parent affected by Huntington's Disease. In reflecting on family-of-origin experiences, clients can also express implied expectations of the future, and future relationships. Case examples are given to illustrate the dynamic processes of identification and fear of abandonment which may present in the clinical setting. Counselor recognition of these processes can illuminate and inform counseling practice.

  5. Abnormalities in the tricarboxylic Acid cycle in Huntington disease and in a Huntington disease mouse model.

    Science.gov (United States)

    Naseri, Nima N; Xu, Hui; Bonica, Joseph; Vonsattel, Jean Paul G; Cortes, Etty P; Park, Larry C; Arjomand, Jamshid; Gibson, Gary E

    2015-06-01

    Glucose metabolism is reduced in the brains of patients with Huntington disease (HD). The mechanisms underlying this deficit, its link to the pathology of the disease, and the vulnerability of the striatum in HD remain unknown. Abnormalities in some of the key mitochondrial enzymes involved in glucose metabolism, including the pyruvate dehydrogenase complex (PDHC) and the tricarboxylic acid (TCA) cycle, may contribute to these deficits. Here, activities for these enzymes and select protein levels were measured in human postmortem cortex and in striatum and cortex of an HD mouse model (Q175); mRNA levels encoding for these enzymes were also measured in the Q175 mouse cortex. The activities of PDHC and nearly all of the TCA cycle enzymes were dramatically lower (-50% to 90%) in humans than in mice. The activity of succinate dehydrogenase increased with HD in human (35%) and mouse (23%) cortex. No other changes were detected in the human HD cortex or mouse striatum. In Q175 cortex, there were increased activities of PDHC (+12%) and aconitase (+32%). Increased mRNA levels for succinyl thiokinase (+88%) and isocitrate dehydrogenase (+64%) suggested an upregulation of the TCA cycle. These patterns of change differ from those reported in other diseases, which may offer unique metabolic therapeutic opportunities for HD patients.

  6. Pluripotent hybrid stem cells from transgenic Huntington's disease monkey.

    Science.gov (United States)

    Laowtammathron, Chuti; Chan, Anthony W S

    2013-01-01

    Huntington's disease (HD) is a devastating disease that currently has no cure. Transgenic HD monkeys have developed key neuropathological and cognitive behavioral impairments similar to HD patients. Thus, pluripotent stem cells derived from transgenic HD monkeys could be a useful comparative model for clarifying HD pathogenesis and developing novel therapeutic approaches, which could be validated in HD monkeys. In order to create personal pluripotent stem cells from HD monkeys, here we present a tetraploid technique for deriving pluripotent hybrid HD monkey stem cells.

  7. Striatal degeneration impairs language learning: evidence from Huntington's disease.

    Science.gov (United States)

    De Diego-Balaguer, R; Couette, M; Dolbeau, G; Dürr, A; Youssov, K; Bachoud-Lévi, A-C

    2008-11-01

    Although the role of the striatum in language processing is still largely unclear, a number of recent proposals have outlined its specific contribution. Different studies report evidence converging to a picture where the striatum may be involved in those aspects of rule-application requiring non-automatized behaviour. This is the main characteristic of the earliest phases of language acquisition that require the online detection of distant dependencies and the creation of syntactic categories by means of rule learning. Learning of sequences and categorization processes in non-language domains has been known to require striatal recruitment. Thus, we hypothesized that the striatum should play a prominent role in the extraction of rules in learning a language. We studied 13 pre-symptomatic gene-carriers and 22 early stage patients of Huntington's disease (pre-HD), both characterized by a progressive degeneration of the striatum and 21 late stage patients Huntington's disease (18 stage II, two stage III and one stage IV) where cortical degeneration accompanies striatal degeneration. When presented with a simplified artificial language where words and rules could be extracted, early stage Huntington's disease patients (stage I) were impaired in the learning test, demonstrating a greater impairment in rule than word learning compared to the 20 age- and education-matched controls. Huntington's disease patients at later stages were impaired both on word and rule learning. While spared in their overall performance, gene-carriers having learned a set of abstract artificial language rules were then impaired in the transfer of those rules to similar artificial language structures. The correlation analyses among several neuropsychological tests assessing executive function showed that rule learning correlated with tests requiring working memory and attentional control, while word learning correlated with a test involving episodic memory. These learning impairments significantly

  8. Long-term outcome of presymptomatic testing in Huntington disease

    OpenAIRE

    Gargiulo, Marcela; Lejeune, Séverine; Tanguy, Marie-Laure; Lahlou-Laforêt, Khadija; Faudet, Anne; Cohen, David; Feingold, Josué; Durr, Alexandra

    2008-01-01

    Our study on long-term outcome of presymptomatic testing for Huntington disease had two aims: the comparison of the psychological well-being and social adjustment of carriers and non-carriers of the mutation, and the identification of psychological determinants to improve care/support of testees. We performed a cross-sectional study of 351 persons who underwent presymptomatic testing. Those who had motor signs were excluded from the comparison of asymptomatic carrier and non-carriers. A struc...

  9. Biological assessment of streams in the Indianapolis Metropolitan Area, Indiana, 1999-2001

    Science.gov (United States)

    Voelker, David C.

    2004-01-01

    .2 (good) to 8.0 (poor) for the tributary sites. The lowest scores among the White River sites were at the Nora site, indicating the best water-quality conditions were where the White River enters Marion County. The highest HBI scores were at the Morris and Harding sites, indicating the least-favorable water-quality conditions of all the White River sites. Of the tributary sites, HBI scores for Buck, Eagle, and Williams Creeks indicate fair water-quality conditions; HBI scores for Pleasant Run and Pogues Run were the highest, indicating relatively poor water-quality conditions. On the White River, the highest Invertebrate Community Index (ICI) scores, which indicate the best benthic-invertebrate conditions, were at the Nora site. Conditions were fair to poor in the downtown Indianapolis area; ICI scores indicate slight improvement in the downstream reaches of the study area. Of the tributary sites, Buck Creek was the only site with ICI scores indicating exceptional water quality. Williams Creek ICI scores indicate good water quality; the remaining tributary-site scores reflect fair conditions. A total of 74 species and 3 hybrids of fish were identified during the study period. The Cyprinidae (carps and minnows) was the largest group of fish identified and consisted of more than half of all fish collected. The most numerous species was the central stoneroller (Campostoma anomalum), which accounted for almost 25 percent of the fish identified. Two nonnative species, the koi carp (Cyprinus carpio) and the western mosquitofish (Gambusia affinis), and one species classified as an Indiana species of special concern, the northern studfish (Fundulus catenatus), also were collected during the study. Indiana Index of Biotic Integrity (IBI) and Ohio Index of Biotic Integrity scores were calculated to show the condition of the fish communities at each site. Results of the Indiana IBI calculations showed no apparent differences in scores among the Wh

  10. The structural involvement of the cingulate cortex in premanifest and early Huntington's disease.

    Science.gov (United States)

    Hobbs, Nicola Z; Pedrick, Amy V; Say, Miranda J; Frost, Chris; Dar Santos, Rachelle; Coleman, Allison; Sturrock, Aaron; Craufurd, David; Stout, Julie C; Leavitt, Blair R; Barnes, Josephine; Tabrizi, Sarah J; Scahill, Rachael I

    2011-08-01

    The impact of Huntington's disease neuropathology on the structure of the cingulate is uncertain, with evidence of both cortical enlargement and atrophy in this structure in early clinical disease. We sought to determine differences in cingulate volume between premanifest Huntington's disease and early Huntington's disease groups compared with controls using detailed manual measurements. Thirty controls, 30 subjects with premanifest Huntington's disease, and 30 subjects with early Huntington's disease were selected from the Vancouver site of the TRACK-HD study. Subjects underwent 3 Tesla magnetic resonance imaging and motor, cognitive, and neuropsychiatric assessment. The cingulate was manually delineated and subdivided into rostral, caudal, and posterior segments. Group differences in volume and associations with performance on 4 tasks thought to utilize cingulate function were examined, with adjustment for appropriate covariates. Cingulate volumes were, on average, 1.7 mL smaller in early Huntington's disease (P=.001) and 0.9 mL smaller in premanifest Huntington's disease (P=.1) compared with controls. Smaller volumes in subsections of the cingulate were associated with impaired recognition of negative emotions (P=.04), heightened depression (P=.009), and worse visual working memory performance (P=.01). There was no evidence of associations between volume and ability on a performance-monitoring task. This study disputes previous findings of enlargement of the cingulate cortex in Huntington's disease and instead suggests that the cingulate undergoes structural degeneration during early Huntington's disease with directionally consistent, nonsignificant differences seen in premanifest Huntington's disease. Cingulate atrophy may contribute to deficits in mood, emotional processing, and visual working memory in Huntington's disease.

  11. Preimplantation genetic diagnosis for Huntington's disease with exclusion testing.

    Science.gov (United States)

    Sermon, Karen; De Rijcke, Martine; Lissens, Willy; De Vos, Anick; Platteau, Peter; Bonduelle, Maryse; Devroey, Paul; Van Steirteghem, André; Liebaers, Inge

    2002-10-01

    Huntington's disease is an autosomal dominant, late-onset disorder, for which the gene and the causative mutation have been known since 1993. Some at-risk patients choose for presymptomatic testing and can make reproductive choices accordingly. Others however, prefer not to know their carrier status, but may still wish to prevent the birth of a carrier child. For these patients, exclusion testing after prenatal sampling has been an option for many years. A disadvantage of this test is that unaffected pregnancies may be terminated if the parent at risk (50%) has not inherited the grandparental Huntington gene, leading to serious moral and ethical objections. As an alternative, preimplantation genetic diagnosis (PGD) on embryos obtained in vitro may be proposed, after which only embryos free of risk are replaced. Embryos can then be selected, either by the amplification of the CAG repeat in the embryos without communicating results to the patients (ie non-disclosure testing), which brings its own practical and moral problems, or exclusion testing. We describe here the first PGD cycles for exclusion testing for Huntington's disease in five couples. Three couples have had at least one PGD cycle so far. One pregnancy ensued and a healthy female baby was delivered.

  12. iPSC-based drug screening for Huntington's disease.

    Science.gov (United States)

    Zhang, Ningzhe; Bailus, Barbara J; Ring, Karen L; Ellerby, Lisa M

    2016-05-01

    Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder, caused by an expansion of the CAG repeat in exon 1 of the huntingtin gene. The disease generally manifests in middle age with both physical and mental symptoms. There are no effective treatments or cures and death usually occurs 10-20 years after initial symptoms. Since the original identification of the Huntington disease associated gene, in 1993, a variety of models have been created and used to advance our understanding of HD. The most recent advances have utilized stem cell models derived from HD-patient induced pluripotent stem cells (iPSCs) offering a variety of screening and model options that were not previously available. The discovery and advancement of technology to make human iPSCs has allowed for a more thorough characterization of human HD on a cellular and developmental level. The interaction between the genome editing and the stem cell fields promises to further expand the variety of HD cellular models available for researchers. In this review, we will discuss the history of Huntington's disease models, common screening assays, currently available models and future directions for modeling HD using iPSCs-derived from HD patients. This article is part of a Special Issue entitled SI: PSC and the brain. Copyright © 2015 Elsevier B.V. All rights reserved.

  13. Informativeness of Early Huntington Disease Signs about Gene Status.

    Science.gov (United States)

    Oster, Emily; Eberly, Shirley W; Dorsey, E Ray; Kayson-Rubin, Elise; Oakes, David; Shoulson, Ira

    2015-01-01

    The cohort-level risk of Huntington disease (HD) is related to the age and symptom level of the cohort, but this relationship has not been made precise. To predict the evolving likelihood of carrying the Huntington disease (HD) gene for at-risk adults using age and sign level. Using data from adults with early signs and symptoms of HD linked to information on genetic status, we use Bayes' theorem to calculate the probability that an undiagnosed individual of a certain age and sign level has an expanded CAG repeat. Both age and sign levels have substantial influence on the likelihood of HD onset, and the probability of eventual diagnosis changes as those at risk age and exhibit (or fail to exhibit) symptoms. For example, our data suggest that in a cohort of individuals age 26 with a Unified Huntington's Disease Rating Scale (UHDRS) motor score of 7-10 70% of them will carry the HD mutation. For individuals age 56, the same motor score suggests only a 40% chance of carrying the mutation. Early motor signs of HD, overall and the chorea subscore, were highly predictive of disease onset at any age. However, body mass index (BMI) and cognitive performance scores were not as highly predictive. These results suggest that if researchers or clinicians are looking for early clues of HD, it may be more foretelling to look at motor rather than cognitive signs. Application of similar approaches could be used with other adult-onset genetic conditions.

  14. Contribution of imaging studies and neuro physiologic investigations to the diagnosis of Huntington`s chorea; L`imagerie medicale et les explorations neuro-physiologiques dans le diagnostic de la choree de Huntington

    Energy Technology Data Exchange (ETDEWEB)

    Paquet, J.M.; Turpin, J.CI. [Centre Hospitalier Universitaire, 51 - Reims (France)

    1997-05-01

    Although Huntington`s disease was described in 1872, its diagnosis continues to rest on clinical grounds. Recently developed techniques for imaging the brain (computed tomography and magnetic resonance imaging) or studying its function (single photon emission computed tomography and positron emission tomography) have demonstrated only non specific abnormalities at the early stages of the disease, thus failing to improve the pre-symptomatic diagnosis. Neuro-physiologic investigations (evoked potentials, electromyogram, electroencephalogram) have been similarly unrewarding. Investigations are useful only as an laid to the differential diagnosis. Molecular biology technology is the only available tool for identifying high-risk individuals and establishing a definitive diagnosis of Huntington`s disease. (authors). 10 refs.

  15. Mexican Americans and the American Nation: A Response to Professor Huntington

    Science.gov (United States)

    Telles, Edward

    2006-01-01

    This essay is based on a talk I delivered at Texas A&M University on December 10, 2005, in response to an earlier lecture at the university by Professor Samuel P. Huntington. It relies on social science evidence to first address Huntington's contention that Mexicans are overwhelming American borders. It then turns to evidence that Mexican…

  16. PROBLEMS OF MODERNIZATION IN THE WORKS OF S. HUNTINGTON: THEORY AND PRACTICE

    Directory of Open Access Journals (Sweden)

    Britikova E. A.

    2016-06-01

    Full Text Available The article discusses the interpretation of the mechanisms of modernization of the American scientist - Samuel Huntington, which sees modernization as a complex process with a very uncertain result. As a representative of the multilinear approach, Samuel Huntington proves the uniqueness of the modernization paths of each individual national system

  17. Huntington Disease: A Case Study of Early Onset Presenting as Depression

    Science.gov (United States)

    Duesterhus, Pia; Schimmelmann, Benno Graf; Wittkugel, Oliver; Schulte-Markwort, Michael

    2004-01-01

    Huntington disease is a dominantly inherited, neurodegenerative disease characterized by choreiform movement disturbances and dementia, usually with adult onset. The rare juvenile-onset Huntington disease differs from the adult phenotype. A case presenting twice, at age 10 with all the signs of a major depression and age 14 with mutism and…

  18. Huntington disease and Huntington disease-like in a case series from Brazil.

    Science.gov (United States)

    Castilhos, R M; Souza, A F D; Furtado, G V; Gheno, T C; Silva, A L; Vargas, F R; Lima, M-A F D; Barsottini, O; Pedroso, J L; Godeiro, C; Salarini, D; Pereira, E T; Lin, K; Toralles, M-B; Saute, J A M; Rieder, C R; Quintas, M; Sequeiros, J; Alonso, I; Saraiva-Pereira, M L; Jardim, L B

    2014-10-01

    The aim of this study was to identify the relative frequency of Huntington's disease (HD) and HD-like (HDL) disorders HDL1, HDL2, spinocerebellar ataxia type 2 (SCA2), SCA17, dentatorubral-pallidoluysian degeneration (DRPLA), benign hereditary chorea, neuroferritinopathy and chorea-acanthocytosis (CHAC), in a series of Brazilian families. Patients were recruited in seven centers if they or their relatives presented at least chorea, besides other findings. Molecular studies of HTT, ATXN2, TBP, ATN1, JPH3, FTL, NKX2-1/TITF1 and VPS13A genes were performed. A total of 104 families were ascertained from 2001 to 2012: 71 families from South, 25 from Southeast and 8 from Northeast Brazil. There were 93 HD, 4 HDL2 and 1 SCA2 families. Eleven of 104 index cases did not have a family history: 10 with HD. Clinical characteristics were similar between HD and non-HD cases. In HD, the median expanded (CAG)n (range) was 44 (40-81) units; R(2) between expanded HTT and age-at-onset (AO) was 0.55 (p=0.0001, Pearson). HDL2 was found in Rio de Janeiro (2 of 9 families) and Rio Grande do Sul states (2 of 68 families). We detected HD in 89.4%, HDL2 in 3.8% and SCA2 in 1% of 104 Brazilian families. There were no cases of HDL1, SCA17, DRPLA, neuroferritinopathy, benign hereditary chorea or CHAC. Only six families (5.8%) remained without diagnosis.

  19. Flood-inundation maps for White River at Petersburg, Indiana

    Science.gov (United States)

    Fowler, Kathleen K.

    2015-08-20

    Digital flood-inundation maps for a 7.7-mile reach of the White River at Petersburg, Indiana, were created by the U.S. Geological Survey (USGS), in cooperation with the Indiana Office of Community and Rural Affairs. The inundation maps, which can be accessed through the USGS Flood Inundation Mapping Science Web site at http://water.usgs.gov/osw/flood_inundation/, depict estimates of the areal extent and depth of flooding corresponding to selected water levels (stages) at the USGS streamgage at White River at Petersburg, Ind. (03374000). Near-real-time stages at this streamgage may be obtained from the USGS National Water Information System at http://waterdata.usgs.gov/ or the National Weather Service (NWS) Advanced Hydrologic Prediction Service at http:/water.weather.gov/ahps/, which also forecasts flood hydrographs at this site (PTRI3).

  20. Biology teachers' attitudes toward and use of Indiana's evolution standards

    Science.gov (United States)

    Donnelly, Lisa A.; Boone, William J.

    2007-02-01

    This study examines the relationship between biology teachers' evolution teaching practices and their regard and use of Indiana state evolution standards. A survey developed by the authors contained five subscales: use of standards; attitude toward standards; attitude toward evolution standards; evolution teaching practices; and demographic information. This survey was administered to 229 Indiana biology teachers. Data were analyzed using the Rasch model to convert Likert rating scale data into ratio data used for parametric analyses. Correlation analysis revealed significant relationships between use of standards and attitude toward standards, attitude toward standards and evolution teaching practices, and attitude toward evolution standards and days teaching evolution. These findings suggest practical relationships between standards use, teachers' regard of standards, and evolution teaching practices.

  1. A state-based model of prevention: Indiana's example.

    Science.gov (United States)

    Agley, Jon; Gassman, Ruth

    2008-04-01

    Public health officials in the United States have battled alcohol, tobacco, and other drug (ATOD) use among adolescents for the past few decades, but only in 2002 did they begin to see a decline in rates of use. ATOD use and abuse are associated with numerous problems, including criminal behavior and increased adolescent morbidity and mortality rates. Researchers have sought to identify best-practice procedures for ATOD prevention; the state of Indiana has a strong ATOD prevention system in place that has the potential to serve as a model for other U.S. localities because of its best-practice approach to public health services. This article outlines the activities of the Indiana Prevention Resource Center to provide an example to strengthen public health professionals' ability to prevent ATOD use and abuse and to provide for a healthy adolescent population.

  2. Measurement of caudate nucleus area - a more accurate measurement for Huntington's disease

    Energy Technology Data Exchange (ETDEWEB)

    Wardlaw, J.M.; Abernethy, L.J. (Royal Infirmary, London (United Kingdom). Dept. of Radiology); Sellar, R.J. (Western General Hospital, Edinburgh (United Kingdom). Dept. of Neuroradiology)

    1991-08-01

    Caudate nucleus atrophy occurs in Huntington's disease and methods of measuring this have been described using axial CT, but these are indirect and lack sensitivity. We measured caudate nucleus area (blind to the subjects' clinical state) in 30 subjects with or at risk of Huntington's disease, and in 100 normal age matched controls. Fifteen subjects with established symptomatic Huntington's disease, 3 with early symptoms, and 3 presymptomatic subjects (2 showing a high probability for the Huntington's disease gene on genetic testing, and one who has since developed symptoms) were correctly identified. Three normal (gene negative) family members were also correctly identified. Outcome is awaited in 6. CT caudate area measurement is simple and reproducible and we have found it to be a useful confirmatory test for Huntington's disease. (orig.).

  3. Do Wind Turbines Affect Weather Conditions?: A Case Study in Indiana

    Directory of Open Access Journals (Sweden)

    Meghan F. Henschen

    2011-01-01

    Full Text Available Wind turbines are becoming increasingly widespread in the United States as the world looks for cleaner sources of energy. Scientists, policymakers, and citizens have strong opinions regarding the positive and negative effects of wind energy projects, and there is a great deal of misinformation about wind energy circulating on the Web and other media sources. The purpose of this study is to gain a better understanding of how the rotation of hundreds of turbines can influence local weather conditions within a wind farm and in the surrounding areas. This experiment measures temperature, atmospheric pressure, wind speed, wind direction, relative humidity, and evaporation with five weather instruments at Meadow Lake Wind Farm located in White, Jasper, and Benton Counties, Indiana, from November 4 through November 18, 2010. The data show that as wind passes throughout the wind farm, the air warms during the overnight and early morning hours and cools during daytime hours. Observed lower humidity rates and higher evaporation rates downwind also demonstrate that the air dries out as it travels through the wind farm. Further research over multiple seasons is necessary to examine the effects of warmer nighttime temperatures and drier conditions progressively downwind of the installation. Nevertheless, wind turbines did not negatively affect local weather patterns in our small-scale research and may actually prevent frost, which could have important positive implications for farmers by potentially prolonging the growing season.

  4. Liquefaction hazard for the region of Evansville, Indiana

    Science.gov (United States)

    Haase, Jennifer S.; Choi, Yoon S.; Nowack, Robert L.; Cramer, Chris H.; Boyd, Oliver S.; Bauer, Robert A.

    2011-01-01

    We calculated liquefaction potential index for a grid of sites in the Evansville, Indiana area for two scenario earthquakes-a magnitude 7.7 in the New Madrid seismic zone and a M6.8 in the Wabash Valley seismic zone. For the latter event, peak ground accelerations range from 0.13 gravity to 0.81 gravity, sufficiently high to be of concern for liquefaction.

  5. Fresh Market Tomato Pruning Trial for Northern Indiana, 2001

    OpenAIRE

    2001-01-01

    Pruning tomatoes is known to increase average fruit size and decrease total yield. Effects on marketable yield and early yield vary among cultivars and with the degree of pruning. This trial was conducted to evaluate a range of pruning treatments on two cultivars grown in the Midwest: Mountain Spring and Florida 91. Florida 91 has a larger vine and is later-maturing than Mountain Spring. The trial was conducted at the Pinney-Purdue Agricultural Center in Wanatah, Indiana.

  6. Determination of Practical ESALs Per Truck Values on Indiana Roads

    OpenAIRE

    2000-01-01

    The Indiana Department of Transportation (INDOT) has been using ESAL (Equivalent Single Axle Load) values for pavement design. The current ESAL values were obtained in the late 1970. However, truck deregulation and higher allowable maximum loads have increased the 1970's values. Updated ESAL values are needed for better road designs. The 1998 and 1999 traffic data from the all Weight-in-Motion (WIM) stations were obtained and used to compute updated ESAL values for multiple unit trucks (Class...

  7. Organic geochemistry in Pennsylvanian tidally influenced sediments from SW Indiana

    Energy Technology Data Exchange (ETDEWEB)

    Mastalerz, M.; Kvale, E.P.; Stankiewicz, B.A.; Portle, K. [Indiana University, Bloomington, IN (United States). Indiana Geological Survey

    1999-05-01

    Tidal rhythmites are vertically stacked small-scale sedimentary structures that record daily variations in tidal current energy and are known to overlie some low-sulfur coals in the Illinois Basin. Tidal rhythmites from the Pennsylvanian Brazil Formation in Indiana were analyzed sedimentologically, petrographically, and geochemically in order to understand the character and distribution of organic matter preserved in an environment of daily interactions between marine and fresh waters.

  8. Clinical and counselling implications of preimplantation genetic diagnosis for Huntington's disease in the UK.

    Science.gov (United States)

    Lashwood, A; Flinter, F

    2001-01-01

    Huntington's disease is an autosomal dominant neurodegenerative disorder that usually occurs in adult life. Individuals at risk can have a gene test before the onset of symptoms, and prenatal diagnosis is available. Preimplantation genetic diagnosis (PGD) for Huntington's disease is now available for couples in whom one partner has the gene for Huntington's disease. A licence to practise PGD is required from the Human Fertilisation and Embryology Authority, and there are several complex issues relating to PGD for Huntington's disease that require consideration. The partner of the Huntington's disease gene carrier should have a presymptomatic test to ensure accuracy in a PGD cycle. There should be a delay between blood sampling and testing for Huntington's disease to allow time for reflection and withdrawal from testing. All PGD treatment has an associated risk of misdiagnosis. If confirmatory prenatal testing is not undertaken after a successful PGD cycle, no confirmation of diagnosis will be obtained at birth. Guidelines indicate that individuals who are at risk cannot be tested before 18 years. There is concern over the ability of a child or adolescent to make an informed choice about testing before this age. Confirmatory testing at birth after PGD would be in direct contravention of these guidelines. In the UK, the law requires consideration of the welfare of children born after assisted conception treatment. Presenting symptoms of Huntington's disease may affect the parenting abilities of an affected individual. There is a need for an assessment of a patient's current Huntington's disease status and their planned provision of care of children if Huntington's disease affects parenting. It has been necessary to create a detailed working protocol for the management of PGD for Huntington's disease to address these issues.

  9. Current status of PET imaging in Huntington's disease.

    Science.gov (United States)

    Pagano, Gennaro; Niccolini, Flavia; Politis, Marios

    2016-06-01

    To review the developments of recent decades and the current status of PET molecular imaging in Huntington's disease (HD). A systematic review of PET studies in HD was performed. The MEDLINE, Web of Science, Cochrane and Scopus databases were searched for articles in all languages published up to 19 August 2015 using the major medical subject heading "Huntington Disease" combined with text and key words "Huntington Disease", "Neuroimaging" and "PET". Only peer-reviewed, primary research studies in HD patients and premanifest HD carriers, and studies in which clinical features were described in association with PET neuroimaging results, were included in this review. Reviews, case reports and nonhuman studies were excluded. A total of 54 PET studies were identified and analysed in this review. Brain metabolism ([(18)F]FDG and [(15)O]H2O), presynaptic ([(18)F]fluorodopa, [(11)C]β-CIT and [(11)C]DTBZ) and postsynaptic ([(11)C]SCH22390, [(11)C]FLB457 and [(11)C]raclopride) dopaminergic function, phosphodiesterases ([(18)F]JNJ42259152, [(18)F]MNI-659 and [(11)C]IMA107), and adenosine ([(18)F]CPFPX), cannabinoid ([(18)F]MK-9470), opioid ([(11)C]diprenorphine) and GABA ([(11)C]flumazenil) receptors were evaluated as potential biomarkers for monitoring disease progression and for assessing the development and efficacy of novel disease-modifying drugs in premanifest HD carriers and HD patients. PET studies evaluating brain restoration and neuroprotection were also identified and described in detail. Brain metabolism, postsynaptic dopaminergic function and phosphodiesterase 10A levels were proven to be powerful in assessing disease progression. However, no single technique may be currently considered an optimal biomarker and an integrative multimodal imaging approach combining different techniques should be developed for monitoring potential neuroprotective and preventive treatment in HD.

  10. Flood-inundation maps for the Yellow River at Plymouth, Indiana

    Science.gov (United States)

    Menke, Chad D.; Bunch, Aubrey R.; Kim, Moon H.

    2016-11-16

    Digital flood-inundation maps for a 4.9-mile reach of the Yellow River at Plymouth, Indiana (Ind.), were created by the U.S. Geological Survey (USGS) in cooperation with the Indiana Office of Community and Rural Affairs. The flood-inundation maps, which can be accessed through the USGS Flood Inundation Mapping Science Web site at http://water.usgs.gov/osw/flood_inundation/, depict estimates of the areal extent and depth of flooding corresponding to selected water levels (stages) at the USGS streamgage 05516500, Yellow River at Plymouth, Ind. Current conditions for estimating near-real-time areas of inundation using USGS streamgage information may be obtained on the Internet at http://waterdata.usgs.gov/in/nwis/uv?site_no=05516500. In addition, information has been provided to the National Weather Service (NWS) for incorporation into their Advanced Hydrologic Prediction Service (AHPS) flood-warning system (http:/water.weather.gov/ahps/). The NWS AHPS forecasts flood hydrographs at many sites that are often collocated with USGS streamgages, including the Yellow River at Plymouth, Ind. NWS AHPS-forecast peak-stage information may be used in conjunction with the maps developed in this study to show predicted areas of flood and forecasts of flood hydrographs at this site.For this study, flood profiles were computed for the Yellow River reach by means of a one-dimensional step-backwater model. The hydraulic model was calibrated by using the current stage-discharge relations at the Yellow River streamgage, in combination with the flood-insurance study for Marshall County (issued in 2011). The calibrated hydraulic model was then used to determine eight water-surface profiles for flood stages at 1-foot intervals referenced to the streamgage datum and ranging from bankfull to the highest stage of the current stage-discharge rating curve. The 1-percent annual exceedance probability flood profile elevation (flood elevation with recurrence intervals within 100 years) is within

  11. El trabajo interdisciplinar en la enfermedad de Huntington

    OpenAIRE

    Fernández Hawrylak, María; Grau Rubio, Claudia; Hernández Lozano, David; Fernández Sastre, Beatriz

    2014-01-01

    Se argumenta la importancia del trabajo en equipo en la atención de las personas afectadas por la Enfermedad de Huntington y de sus familias, y se describen las principales funciones de los distintos profesionales que han de cubrir sus necesidades en cada una de las etapas de la enfermedad en función de las alteraciones y secuelas. Siguiendo esta premisa, se presenta el trabajo de intervención basado en la complementariedad de distintos profesionales que atienden y cuidan a las personas afect...

  12. ENFERMEDAD DE HUNTINGTON: MODELOS EXPERIMENTALES Y PERSPECTIVAS TERAPÉUTICAS

    OpenAIRE

    TERESA SERRANO SÁNCHEZ; LISETTE BLANCO LEZCANO; ROCÍO GARCÍA MINET; ESTEBAN ALBERTI AMADOR; IVÁN DÍAZ ARMESTO; NANCY PAVÓN FUENTE; LOURDES LORIGADOS PEDRE; MARÍA ELENA GONZÁLEZ FRAGUELA; JORGE FELIPE MONTERO LEÓN; LISIS MARTÍNEZ MARTÍ; MARÍA DE LOS ANGELES ROBINSON AGRAMONTE; LILIANA FRANCIS TURNER

    2011-01-01

    La enfermedad de Huntington (EH) es un trastorno degenerativo de Weiss de origen hereditario. Hasta el momento no existe un tratamiento efectivo para la enfermedad que inexorablemente después de transcurridos 15 a 20 años, evoluciona hacia incapa- cidad total o muerte. En este trabajo se revisan las características clínicas y morfológicas de la EH y los modelos experimentales más utilizados para su estudio tomando como fuente, artículos indexados en la base de datos Medline publicados en los ...

  13. Huntington's disease as caused by 34 CAG repeats.

    Science.gov (United States)

    Andrich, Jürgen; Arning, Larissa; Wieczorek, Stefan; Kraus, Peter H; Gold, Ralf; Saft, Carsten

    2008-04-30

    Huntington's disease (HD) is an autosomal dominantly inherited neurodegenerative disorder caused by an abnormal expansion of a polymorphic stretch of CAG repeats in the coding 5' part of the HD gene on chromosome 4p. Expansions of CAG blocks beyond 35 repeats are associated with the clinical presentation of HD. There is an intermediate range of rare alleles between 27 and 35 CAG repeats with a higher risk for further expansion in subsequent generations. Here, we report a 75-year-old male with clinical features of HD and 34 CAG repeat units.

  14. Polyglutamine Aggregation in Huntington Disease: Does Structure Determine Toxicity?

    Science.gov (United States)

    Hoffner, Guylaine; Djian, Philippe

    2015-12-01

    Huntington disease is a dominantly inherited disease of the central nervous system. The mutational expansion of polyglutamine beyond a critical length produces a toxic gain of function in huntingtin and results in neuronal death. In the course of the disease, expanded huntingtin is proteolyzed, becomes abnormally folded, and accumulates in oligomers, fibrils, and microscopic inclusions. The aggregated forms of the expanded protein are structurally diverse. Structural heterogeneity may explain why polyglutamine-containing aggregates could paradoxically be either toxic or neuroprotective. When defined, the toxic structures could then specifically be targeted by prophylactic or therapeutic drugs aimed at inhibiting polyglutamine aggregation.

  15. Reduced gluconeogenesis and lactate clearance in Huntington's disease

    DEFF Research Database (Denmark)

    Josefsen, Knud; Nielsen, Signe M B; Campos, André

    2010-01-01

    We studied systemic and brain glucose and lactate metabolism in Huntington's disease (HD) patients in response to ergometer cycling. Following termination of exercise, blood glucose increased abruptly in control subjects, but no peak was seen in any of the HD patients (2.0 ± 0.5 vs. 0.0 ± 0.2mM, P...... for gluconeogenesis in HD, possibly contributing to the clinical symptoms of HD. We propose that blood glucose concentration in the recovery from exercise can be applied as a liver function test in HD patients....

  16. The Cambridge MRI database for animal models of Huntington disease.

    Science.gov (United States)

    Sawiak, Stephen J; Morton, A Jennifer

    2016-01-01

    We describe the Cambridge animal brain magnetic resonance imaging repository comprising 400 datasets to date from mouse models of Huntington disease. The data include raw images as well as segmented grey and white matter images with maps of cortical thickness. All images and phenotypic data for each subject are freely-available without restriction from (http://www.dspace.cam.ac.uk/handle/1810/243361/). Software and anatomical population templates optimised for animal brain analysis with MRI are also available from this site.

  17. Mutant Huntingtin Does Not Affect the Intrinsic Phenotype of Human Huntington's Disease T Lymphocytes.

    Science.gov (United States)

    Miller, James R C; Träger, Ulrike; Andre, Ralph; Tabrizi, Sarah J

    2015-01-01

    Huntington's disease is a fatal neurodegenerative condition caused by a CAG repeat expansion in the huntingtin gene. The peripheral innate immune system is dysregulated in Huntington's disease and may contribute to its pathogenesis. However, it is not clear whether or to what extent the adaptive immune system is also involved. Here, we carry out the first comprehensive investigation of human ex vivo T lymphocytes in Huntington's disease, focusing on the frequency of a range of T lymphocyte subsets, as well as analysis of proliferation, cytokine production and gene transcription. In contrast to the innate immune system, the intrinsic phenotype of T lymphocytes does not appear to be affected by the presence of mutant huntingtin, with Huntington's disease T lymphocytes exhibiting no significant functional differences compared to control cells. The transcriptional profile of T lymphocytes also does not appear to be significantly affected, suggesting that peripheral immune dysfunction in Huntington's disease is likely to be mediated primarily by the innate rather than the adaptive immune system. This study increases our understanding of the effects of Huntington's disease on peripheral tissues, while further demonstrating the differential effects of the mutant protein on different but related cell types. Finally, this study suggests that the potential use of novel therapeutics aimed at modulating the Huntington's disease innate immune system should not be extended to include the adaptive immune system.

  18. Mutant Huntingtin Does Not Affect the Intrinsic Phenotype of Human Huntington's Disease T Lymphocytes.

    Directory of Open Access Journals (Sweden)

    James R C Miller

    Full Text Available Huntington's disease is a fatal neurodegenerative condition caused by a CAG repeat expansion in the huntingtin gene. The peripheral innate immune system is dysregulated in Huntington's disease and may contribute to its pathogenesis. However, it is not clear whether or to what extent the adaptive immune system is also involved. Here, we carry out the first comprehensive investigation of human ex vivo T lymphocytes in Huntington's disease, focusing on the frequency of a range of T lymphocyte subsets, as well as analysis of proliferation, cytokine production and gene transcription. In contrast to the innate immune system, the intrinsic phenotype of T lymphocytes does not appear to be affected by the presence of mutant huntingtin, with Huntington's disease T lymphocytes exhibiting no significant functional differences compared to control cells. The transcriptional profile of T lymphocytes also does not appear to be significantly affected, suggesting that peripheral immune dysfunction in Huntington's disease is likely to be mediated primarily by the innate rather than the adaptive immune system. This study increases our understanding of the effects of Huntington's disease on peripheral tissues, while further demonstrating the differential effects of the mutant protein on different but related cell types. Finally, this study suggests that the potential use of novel therapeutics aimed at modulating the Huntington's disease innate immune system should not be extended to include the adaptive immune system.

  19. 76 FR 44591 - Notice of Hearing: Reconsideration of Disapproval of Indiana State Plan Amendments (SPA) 11-011

    Science.gov (United States)

    2011-07-26

    ... Disapproval of Indiana State Plan Amendments (SPA) 11-011 AGENCY: Centers for Medicare & Medicaid Services..., Chicago, IL 60601, to reconsider CMS' decision to disapprove Indiana SPA 11-011. DATES: Closing Date... announces an administrative hearing to reconsider CMS' decision to disapprove Indiana SPA 11-011, which...

  20. The High Cost of Failing to Reform Public Education in Indiana. School Choice Issues in the State

    Science.gov (United States)

    Gottlob, Brian J.

    2006-01-01

    This study documents the public costs of high school dropouts in Indiana, and examines how school choice would provide large public benefits by increasing the graduation rate in Indiana public schools. It calculates the annual cost of high school dropouts in Indiana due to lower state income tax payments, increased reliance on Medicaid, and…

  1. The Hoosier Newsman and the Hooded Order: Indiana Press Reaction to the Ku Klux Klan in the 1920s.

    Science.gov (United States)

    Scharlott, Bradford W.

    Coverage in a sample of ten Indiana daily newspapers was analyzed, documentary evidence was gathered, and interviews with surviving newswriters were conducted to determine how the Indiana press reported the Ku Klux Klan during the 1920s. The study found that Indiana papers gave the Klan, while it was powerful, more favorable coverage than…

  2. 75 FR 42069 - Expansion of Foreign-Trade Zone 152, Burns Harbor, Indiana

    Science.gov (United States)

    2010-07-20

    ... From the Federal Register Online via the Government Publishing Office DEPARTMENT OF COMMERCE Foreign-Trade Zones Board Expansion of Foreign-Trade Zone 152, Burns Harbor, Indiana Pursuant to its... in the Burns Harbor, Indiana, area, within the Chicago Customs and Border Protection port of...

  3. 78 FR 1879 - Endangered and Threatened Wildlife and Plants; Draft Revised Indiana Bat Summer Survey Guidelines

    Science.gov (United States)

    2013-01-09

    ...: Background The Indiana bat was originally listed as in danger of extinction under the Endangered Species... From the Federal Register Online via the Government Publishing Office DEPARTMENT OF THE INTERIOR Endangered and Threatened Wildlife and Plants; Draft Revised Indiana Bat Summer Survey Guidelines AGENCY...

  4. 78 FR 9409 - Endangered and Threatened Wildlife and Plants; Draft Revised Indiana Bat Summer Survey Guidelines

    Science.gov (United States)

    2013-02-08

    ... INFORMATION: Background The Indiana bat was originally listed as in danger of extinction under the Endangered... From the Federal Register Online via the Government Publishing Office DEPARTMENT OF THE INTERIOR Fish and Wildlife Service Endangered and Threatened Wildlife and Plants; Draft Revised Indiana Bat...

  5. Presymptomatic testing for Huntington's disease: a world wide survey. The World Federation of Neurology Research Group on Huntington's Disease.

    OpenAIRE

    1993-01-01

    World wide data on presymptomatic testing for Huntington's disease using closely linked DNA markers show that 1479 persons at risk received completed test results up to the end of 1991. Testing has been carried out in 19 countries, with at least 88 centres involved, and numbers have levelled off after a peak in 1990. Only 5% of those at risk have been tested in six countries with the longest established programmes. Continued monitoring of international data will be of value in assessing the s...

  6. Coal Fields - COAL_NCRDS_IN: Coal Resource Data in Indiana, Derived from the National Coal Resource Data System (Indiana Geological Survey, Point Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — This ESRI ArcInfo point shapefile depicts the location of 9,117 public point-source coal resource data points in Indiana. This shapefile includes location and coal...

  7. Recreational Areas - RECREATIONAL_FACILITIES_IDNR_IN: Outdoor Recreational Facilities in Indiana (Indiana Department of Natural Resources, 1:24,000, Point Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — NOTE: The original data set named RECFACILITIES_DNR_OR.SHP was provided to Indiana Geological Survey personnel on August 14, 2003, by Mr. Michael P. Martin, GIS...

  8. Sewerage Treatment Plants - WASTE_TREATMENT_STORAGE_DISPOSAL_IDEM_IN: Treatment, Storage, and Disposal Sites in Indiana (Indiana Department of Environmental Management, Point Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — WASTE_TREATMENT_STORAGE_DISPOSAL_IDEM_IN is a point shapefile that contains treatment, storage, and disposal (TSD) site locations in Indiana, provided by personnel...

  9. Landfills, Hazardous Waste - CONSTRUCTION_DEMOLITION_WASTE_IDEM_IN: Construction and Demoliton Waste Facilities in Indiana (Indiana Department of Environmental Management, Point Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — CONSTRUCTION_DEMOLITION_WASTE_IDEM_IN is a point shapefile that contains construction and demolition waste facility locations in Indiana, provided by personnel of...

  10. Hydrogeology - AQUIFER_SYSTEMS_BEDROCK_IDNR_IN: Bedrock Aquifer Systems of Indiana (Indiana Department of Natural Resources, 1:500,000, Polygon Shapefile)

    Data.gov (United States)

    NSGIC State | GIS Inventory — AQUIFER_SYSTEMS_BEDROCK_IDNR_IN is a polygon shapefile that shows bedrock aquifer systems of the State of Indiana. The source scale of the map depicting the aquifers...

  11. Hydrogeology - AQUIFER_SYSTEMS_UNCONSOLIDATED_IDNR_IN: Unconsolidated Aquifer Systems of Indiana (Indiana Department of Natural Resources, 1:48,000, Polygon Shapefile)

    Data.gov (United States)

    NSGIC State | GIS Inventory — AQUIFER_SYSTEMS_UNCONSOLIDATED_IDNR_IN is a polygon shapefile that shows unconsolidated aquifer systems of the state of Indiana at a scale of 1:48,000. The following...

  12. Geology, Surficial - CLAY_ILITH_IN: Total Thickness of Clay in Indiana, Derived from the iLITH Water-Well Database (Indiana Geological Survey, Grid)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — CLAY_ILITH_IN is a grid that shows total thickness of clay, as derived from logs of water wells in the state of Indiana. (It presents the same data as shown in a...

  13. Geology, Surficial - SAND_ILITH_IN: Total Thickness of Sand and Gravel in Indiana, Derived from the iLITH Water-Well Database (Indiana Geological Survey, Grid)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — SAND_ILITH_IN is a grid that shows total thickness of sand, as derived from logs of water wells in the state of Indiana. (It presents the same data as shown in a...

  14. Geology, Surficial - CLAY_ILITH_PTS_IN: Total Thickness of Clay in Indiana, Derived from the iLITH Water-Well Database (Indiana Geological Survey, Point Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — CLAY_ILITH_PTS_IN is a point shapefile that shows total thickness of clay, as derived from logs of water wells in the state of Indiana. (It presents the source data...

  15. Bathymetric Contours - LAKE_BATHYMETRY_IDNR_IN: Bathymetric Contours for Selected Lakes in Indiana (Indiana Department of Natural Resources, Polygon Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — LAKE_BATHYMETRY_IDNR_IN.SHP provides bathymetric contours for the following 85 lakes in Indiana, with depths calculated from the average shoreline of each lake:...

  16. Hydrogeology - AQUIFER_SYSTEMS_BEDROCK_IDNR_IN: Bedrock Aquifer Systems of Indiana (Indiana Department of Natural Resources, 1:500,000, Polygon Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — AQUIFER_SYSTEMS_BEDROCK_IDNR_IN is a polygon shapefile that shows bedrock aquifer systems of the State of Indiana. The source scale of the map depicting the aquifers...

  17. Vehicle Fleet Information - BMV_LICENSE_BRANCHES_IN: Bureau of Motor Vehicle License Branch Locations in Indiana (Indiana Geological Survey, Point Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — BMV_LICENSE_BRANCHES_IN is a point shapefile showing the locations of 142 branch offices of the Indiana Bureau of Motor Vehicles (BMV). Addresses for each branch...

  18. Hospitals - HOSPITALS_CLINICS_ISDH_IN: Hospitals and Rural Health Clinics in Indiana in 2007 (Indiana State Department of Health, Point Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — HOSPITALS_CLINICS_ISDH_IN is a point shapefile showing the locations of 160 hospitals included in a "Hospital Directory" that appears on a Web page of the Indiana...

  19. Geology, Bedrock - BEDROCK_TOPOGRAPHY_MM36_IN: Bedrock Topography Contours, Indiana (Indiana Geological Survey, 1:500,000, Line Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — Bedrock topography was converted from the original published map, Indiana Geological Survey Miscellaneous Map 36. The contours define the elevation/topography of the...

  20. Trails, Other - TRAILS_IDNR_IN: Public Recreational and Alternative Transportation Trails in Indiana (Indiana Department of Natural Resources, 1:24,000, Line Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — NOTE: The original data set named INTRAILS.SHP was provided to Indiana Geological Survey personnel on March 12, 2002, by Mr. Michael P. Martin, GIS Coordinator,...

  1. Land Use and Land Cover - LAND_COVER_PRESETTLEMENT_IDNR_IN: Generalized Presettlement Vegetation Types of Indiana, Circa 1820 (Indiana Department of Natural Resources, Polygon Shapefile)

    Data.gov (United States)

    NSGIC State | GIS Inventory — LAND_COVER_PRESETTLEMENT_IDNR_IN.SHP is a polygon shapefile showing generalized presettlement vegetation types of Indiana, circa 1820. The work was based on original...

  2. Geology, Bedrock - BEDROCK_TOPOGRAPHY_MM36_IN: Bedrock Topography Contours, Indiana (Indiana Geological Survey, 1:500,000, Line Shapefile)

    Data.gov (United States)

    NSGIC State | GIS Inventory — Bedrock topography was converted from the original published map, Indiana Geological Survey Miscellaneous Map 36. The contours define the elevation/topography of the...

  3. Environmental Monitoring, Other - UNCONSOLIDATED_GAMMA_LOGS_IGS_IN:Natural gamma-ray logs in unconsolidated sediments in Indiana (Indiana Geological Survey, 1:100,000, Point Shapefile)

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — UNCONSOLIDATED_GAMMA_LOGS_IGS_IN.shp shows the locations of the Indiana Geological Survey's gamma-logs that were taken from 1978 to 2005. This shapefile includes...

  4. Inventory and Evaluation of Cultural Resources, Bolsa Chica Mesa and Huntington Beach Mesa, Orange County, California

    Science.gov (United States)

    1989-09-30

    1615 Relacl6n de la Jornada que Hizo el General Sevastian Vizcayno al Descubrimiento de las Californias el Aho de 1602 por Mandado del Segor...California Press, Berkeley. Prosch, R. R. 1978 Catalog of Fossil Hominids of North America . Fischer. New York and Stu’tf-ar t.7 Ricketts, E. F., J

  5. Polymorphisms in the CAG repeat--a source of error in Huntington disease DNA testing.

    Science.gov (United States)

    Yu, S; Fimmel, A; Fung, D; Trent, R J

    2000-12-01

    Five of 400 patients (1.3%), referred for Huntington disease DNA testing, demonstrated a single allele on CAG alone, but two alleles when the CAG + CCG repeats were measured. The PCR assay failed to detect one allele in the CAG alone assay because of single-base silent polymorphisms in the penultimate or the last CAG repeat. The region around and within the CAG repeat sequence in the Huntington disease gene is a hot-spot for DNA polymorphisms, which can occur in up to 1% of subjects tested for Huntington disease. These polymorphisms may interfere with amplification by PCR, and so have the potential to produce a diagnostic error.

  6. DIGITAL FLOOD INSURANCE RATE MAP DATABASE, FRANKLIN COUNTY, INDIANA (AND INCORPORATED AREAS)

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — The Digital Flood Insurance Rate Map (DFIRM) Database depicts flood risk information and supporting data used to develop the risk data. The primary risk...

  7. DIGITAL FLOOD INSURANCE RATE MAP DATABASE, VIGO COUNTY, INDIANA (AND INCORPORATED AREAS)

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — The Digital Flood Insurance Rate Map (DFIRM) Database depicts flood risk information and supporting data used to develop the risk data. The primary risk...

  8. DIGITAL FLOOD INSURANCE RATE MAP DATABASE, BARTHOLOMEW COUNTY, INDIANA (AND INCORPORATED AREAS)

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — The Digital Flood Insurance Rate Map (DFIRM) Database depicts flood risk information and supporting data used to develop the risk data. The primary risk...

  9. DIGITAL FLOOD INSURANCE RATE MAP DATABASE, STEUBEN COUNTY, INDIANA (AND INCORPORATED AREAS)

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — The Digital Flood Insurance Rate Map (DFIRM) Database depicts flood risk information and supporting data used to develop the risk data. The primary risk...

  10. DIGITAL FLOOD INSURANCE RATE MAP DATABASE, UNION COUNTY, INDIANA (AND INCORPORATED AREAS)

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — The Digital Flood Insurance Rate Map (DFIRM) Database depicts flood risk information and supporting data used to develop the risk data. The primary risk...

  11. DIGITAL FLOOD INSURANCE RATE MAP DATABASE, HOWARD COUNTY, INDIANA (AND INCORPORATED AREAS)

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — The Digital Flood Insurance Rate Map (DFIRM) Database depicts flood risk information and supporting data used to develop the risk data. The primary risk...

  12. DIGITAL FLOOD INSURANCE RATE MAP DATABASE, LAGRANGE COUNTY, INDIANA (AND INCORPORATED AREAS)

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — The Digital Flood Insurance Rate Map (DFIRM) Database depicts flood risk information and supporting data used to develop the risk data. The primary risk...

  13. Assessing the value of collaboration in tourism networks: A case study of Elkhart County, Indiana

    DEFF Research Database (Denmark)

    Zach, Florian; Racherla, Pradeep

    2011-01-01

    This study explores the determinants of perceived value derived from interorganizational collaborations in a tourism destination. The authors propose a theoretical model of perceived value drawing upon the rich stream of literature related to strategic collaborations and interorganizational...

  14. DIGITAL FLOOD INSURANCE RATE MAP DATABASE, OHIO COUNTY, INDIANA (AND INCORPORATED AREAS)

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — The Digital Flood Insurance Rate Map (DFIRM) Database depicts flood risk information and supporting data used to develop the risk data. The primary risk...

  15. DIGITAL FLOOD INSURANCE RATE MAP DATABASE, MORGAN COUNTY, INDIANA (AND INCORPORATED AREAS)

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — The Digital Flood Insurance Rate Map (DFIRM) Database depicts flood risk information and supporting data used to develop the risk data. The primary risk...

  16. DIGITAL FLOOD INSURANCE RATE MAP DATABASE, WARRICK COUNTY, INDIANA (AND INCORPORATED AREAS)

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — The Digital Flood Insurance Rate Map (DFIRM) Database depicts flood risk information and supporting data used to develop the risk data. The primary risk...

  17. An Archeological Overview and Management Plan for the Newport Army Ammunition Plant, Vermillion County, Indiana

    Science.gov (United States)

    1984-05-21

    lies within the "warm temperate montane moist for- est" bioclimatic formation (Sawyer and Lindsey 1963). This is the cli- matic zone associated with...summer agricultural villages and winter hunting camps. Villages probably were situated in larger stream valleys, spaced perhaps 30 to 100 miles apart ...University of Wisconsin Press. Sawyer, John 0., Jr., and Alton A. Lindsey. 1963. The Holdridge Bioclimatic Formations of the Eastern and Central United

  18. DIGITAL FLOOD INSURANCE RATE MAP DATABASE, POSEY COUNTY, INDIANA (AND INCORPORATED AREAS)

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — The Digital Flood Insurance Rate Map (DFIRM) Database depicts flood risk information and supporting data used to develop the risk data. The primary risk...

  19. DIGITAL FLOOD INSURANCE RATE MAP DATABASE, JACKSON COUNTY, INDIANA (AND INCORPORATED AREAS)

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — The Digital Flood Insurance Rate Map (DFIRM) Database depicts flood risk information and supporting data used to develop the risk data. The primary risk...

  20. A water-quality assessment of the Feather Creek watershed, Vermillion County, Indiana

    Science.gov (United States)

    Eikenberry, Stephen E.

    1977-01-01

    Chemical quality of surface water within the Feather Creek watershed is generally good. However, fecal bacteria concentrations are high enough to represent a potential problem, especially because of the high water-contact recreation proposed for the future reservoir.

  1. Comparison of evaporation computation methods, Pretty Lake, Lagrange County, northeastern Indiana

    Science.gov (United States)

    Ficke, John F.

    1972-01-01

    Evaporation from Pretty Lake has been computed for a 2%- year period between 1963 and 1965 by the use of an energy budget, mass-transfer parameters, a water budget, a class-A pan, and a computed pan evaporation technique. The seasonal totals for the different methods are within 8 percent of their mean and are within 11 percent of the rate of 79 centimeters (31 inches) per year determined from published maps that are based on evaporation-pan data. Period-by-period differences among the methods are larger than the annual differences, but there is a general agreement among the evaporation hydrographs produced by the different computation methods.

  2. Toxicological evaluation of sediment samples from Burns Harbor, Porter County, Indiana

    Energy Technology Data Exchange (ETDEWEB)

    Ward, J.A.; Pinza, M.R.; Barrows, M.E.; Karls, R.K.; Word, J.Q. [Battelle/Marine Sciences Lab., Sequim, WA (United States)

    1994-05-01

    The US Army Corps of Engineers (USACE) Chicago District is authorized to maintain the water depths in Burns Harbor at navigable levels. In order to maintain these levels, sediments must be dredged and disposed of at approved disposal sites. To make a 404 (b) 1 open-water disposal evaluation, the dredged sediment may be evaluated through a series of toxicological tests to assess its potential for causing an adverse environmental effect. Battelle/Marine Sciences Laboratory (MSL) was contracted by USACE to perform these freshwater toxicity tests. The tests were designed to simulate conditions that organisms living within an aquatic dredged material disposal site might experience during disposal operations, and included both bedded-sediment (solid-phase) and suspended-sediment (elutriate) tests. Test samples were collected by USACE personnel and composited into three test treatments representing potential dredging areas (Management Units {number_sign}1, {number_sign}2, and {number_sign}3). Four toxicological tests were conducted in support of this program. The solid-phase tests included the amphipod, Hyalella azteca, and the midge, Chironomus tentans. The elutriate tests included the fathead minnow, Pimephales promelas, and the daphnid, Daphnia magna. Testing was conducted following standard procedures provided by USACE which are consistent with ASTM protocols and the Evaluation of Dredged Material Proposed for Discharge in Inland and Near Coastal Waters -- Testing Manual (Draft) Inland Testing Manual (EPA/USACE 1993), known as the ``Draft Inland Testing Manual.`` The suitability of sediment representing the management units for open-water disposal was evaluated following the guidelines contained in the Draft Inland Testing Manual.

  3. DIGITAL FLOOD INSURANCE RATE MAP DATABASE, GIBSON COUNTY, INDIANA (AND INCORPORATED AREAS)

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — The Digital Flood Insurance Rate Map (DFIRM) Database depicts flood risk information and supporting data used to develop the risk data. The primary risk...

  4. DIGITAL FLOOD INSURANCE RATE MAP DATABASE, GREENE COUNTY, INDIANA (AND INCORPORATED AREAS)

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — The Digital Flood Insurance Rate Map (DFIRM) Database depicts flood risk information and supporting data used to develop the risk data. The primary risk...

  5. DIGITAL FLOOD INSURANCE RATE MAP DATABASE, WABASH COUNTY, INDIANA (AND INCORPORATED AREAS)

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — The Digital Flood Insurance Rate Map (DFIRM) Database depicts flood risk information and supporting data used to develop the risk data. The primary risk...

  6. DIGITAL FLOOD INSURANCE RATE MAP DATABASE, DUBOIS COUNTY, INDIANA (AND INCORPORATED AREAS)

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — The Digital Flood Insurance Rate Map (DFIRM) Database depicts flood risk information and supporting data used to develop the risk data. The primary risk...

  7. DIGITAL FLOOD INSURANCE RATE MAP DATABASE, JAY COUNTY, INDIANA (AND INCORPORATED AREAS)

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — The Digital Flood Insurance Rate Map (DFIRM) Database depicts flood risk information and supporting data used to develop the risk data. The primary risk...

  8. DIGITAL FLOOD INSURANCE RATE MAP DATABASE, ELKHART COUNTY, INDIANA (AND INCORPORATED AREAS)

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — The Digital Flood Insurance Rate Map (DFIRM) Database depicts flood risk information and supporting data used to develop the risk data. The primary risk...

  9. DIGITAL FLOOD INSURANCE RATE MAP DATABASE, HARRISON COUNTY, INDIANA (AND INCORPORATED AREAS)

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — The Digital Flood Insurance Rate Map (DFIRM) Database depicts flood risk information and supporting data used to develop the risk data. The primary risk...

  10. Hydrologic evaluation of a hypothetical coal-mining site near Chrisney, Spencer County, Indiana

    Science.gov (United States)

    Zogorski, John S.; Ramey, Daniel S.; Lambert, Paul W.; Martin, Jeffrey D.; Warner, Robert E.

    1980-01-01

    Protecting the water resources of the Nation is a major emphasis of the Surface Mining Control and Reclamation Act, PL 95-87. Permanent regulations established for this Act by the Office of Surface Mining (OSM) require the issuance of a permit before mining begins. An application for a mining permit must include an assessment of the hydrologic characteristics of the mining site and adjacent area, and a projection of the potential impacts of mining activities on surface water and ground water. OSM's permanent regulations and guidelines provide little insight on the "how to" aspect of making the required hydrologic assessment. This investigation

  11. The Role of County Surveyors and County Drainage Boards in Addressing Water Quality

    Science.gov (United States)

    Dunn, Mike; Mullendore, Nathan; de Jalon, Silvestre Garcia; Prokopy, Linda Stalker

    2016-06-01

    Water quality problems stemming from the Midwestern U.S. agricultural landscape have been widely recognized and documented. The Midwestern state of Indiana contains tens of thousands of miles of regulated drains that represent biotic communities that comprise the headwaters of the state's many rivers and creeks. Traditional management, however, reduces these waterways to their most basic function as conveyances, ignoring their role in the ecosystem as hosts for biotic and abiotic processes that actively regulate the fate and transport of nutrients and farm chemicals. Novel techniques and practices such as the two-stage ditch, denitrifying bioreactor, and constructed wetlands represent promising alternatives to traditional management approaches, yet many of these tools remain underutilized. To date, conservation efforts and research have focused on increasing the voluntary adoption of practices among agricultural producers. Comparatively little attention has been paid to the roles of the drainage professionals responsible for the management of waterways and regulated drains. To address this gap, we draw on survey responses from 39 county surveyors and 85 drainage board members operating in Indiana. By examining the backgrounds, attitudes, and actions of these individuals, we consider their role in advocating and implementing novel conservation practices.

  12. Presymptomatic diagnosis in Huntington's disease: the Mexican experience.

    Science.gov (United States)

    Alonso, Maria Elisa; Ochoa, Adriana; Sosa, Ana Luisa; Rodríguez, Yaneth; Chávez, Mireya; Boll, Catherine; Yescas, Petra; Macías, Rosario; Rasmussen, Astrid

    2009-12-01

    Huntington's disease (HD) is an autosomal dominant progressive, disabling neurodegenerative disorder, for which there is no effective treatment. Predictive testing (PT) for this illness began in 1986 and by 1993 it became more precise after cloning of the gene and the discovery of a CAG repeat expansion as the underlying cause. The objective of this paper is to illustrate the implementation and results of a PT program in a group of at-risk Mexican individuals with 12 years of follow-up. Our PT program conforms to the guidelines proposed by the International Huntington Association and the HD Working group of the World Federation of Neurology. Seventy-five individuals requested the testing, four of them did not fulfill the inclusion criteria, and five abandoned the program voluntarily before receiving the test results. Therefore, 66 results were delivered to 41 noncarriers and 25 mutation carriers. We did not have any catastrophic event, but 4 individuals with normal results and 11 mutation carriers were depressed. Even if this is a small sample, it is the first report of PT in a Latin-American population in which we have been faced with the same problems referred to in larger series.

  13. Pluripotent Stem Cells Models for Huntington's Disease: Prospects and Challenges

    Institute of Scientific and Technical Information of China (English)

    Richard L. Carter; Anthony W.S. Chan

    2012-01-01

    Pluripotent cellular models have shown great promise in the study of a number of neurological disorders.Several advantages of using a stem cell model include the potential for cells to derive disease relevant neuronal cell types,providing a system for researchers to monitor disease progression during neurogenesis,along with serving as a platform for drug discovery.A number of stem cell derived models have been employed to establish in vitro research models of Huntington's disease that can be used to investigate cellular pathology and screen for drug and cell-based therapies.Although some progress has been made,there are a number of challenges and limitations that must be overcome before the true potential of this research strategy is achieved,In this article we review current stem cell models that have been reported,as well as discuss the issues that impair these studies.We also highlight the prospective application of Huntington's disease stem cell models in the development of novel therapeutic strategies and advancement of personalized medicine.

  14. Progress in studies of gene therapy for Huntington's disease

    Directory of Open Access Journals (Sweden)

    JIN Fan-ying

    2012-06-01

    Full Text Available Huntington's disease (HD is a kind of inherited neurodegenerative disorder characterized by movement problems, cognitive decline and psychiatry disturbance. HD is caused by mutation in gene IT -15 involving the expansion of a trinucleotide (CAG repeat encoding glutamine, which leads to abnormal conformation of huntingtin (Htt protein and finally emerge cytotoxic functions. Currently, HD remains a fatal untreatable disease. Gene therapy for HD discussed in this review is under preclinical studies. Silencing of mutant IT-15 via RNA interference (RNAi or antisense oligonucleotide (ASO has shown some effectiveness in mouse model studies. Increasing the clearance of mutant Htt protein could be achieved by viral-mediated delivery of anti-Htt intrabodies (iAbs or induction of autophagy, and beneficial results have been observed. Ectopic expression of neurotrophic factors, such as nerve growth factor (NGF and brain-derived neurotrophic factor (BDNF, mediated either by viral vectors or transplantation of genetically modified cells, has also been proved to be effective. Other gene-modifying methods aiming at correction of transcriptional dysregulation by histone modification, activation of endogenous neural stem cells, and normalization of calcium signaling and mitochondrial function, are also under intensive research. Gene therapy for Huntington's disease is promising, yet a long way remains from preclinical studies to clinical trials.

  15. Clinical diagnosis and management in early Huntington's disease: a review

    Directory of Open Access Journals (Sweden)

    Schiefer J

    2015-03-01

    Full Text Available Johannes Schiefer,1,* Cornelius J Werner,1,* Kathrin Reetz1,2 1Euregional Huntington Center, 2Jülich Aachen Research Alliance (JARA – Translational Brain Medicine, Department of Neurology, RWTH Aachen University, Aachen, Germany *These authors contributed equally to this work Abstract: This review focuses on clinical diagnosis and both pharmacological and nonpharmacological therapeutic options in early stages of the autosomal dominant inherited neurodegenerative Huntington's disease (HD. The available literature has been reviewed for motor, cognitive, and psychiatric alterations, which are the three major symptom domains of this devastating progressive disease. From a clinical point of view, one has to be aware that the HD phenotype can vary highly across individuals and during the course of the disease. Also, symptoms in juvenile HD can differ substantially from those with adult-onset of HD. Although there is no cure of HD and management is limited, motor and psychiatric symptoms often respond to pharmacotherapy, and nonpharmacological approaches as well as supportive care are essential. International treatment recommendations based on study results, critical statements, and expert opinions have been included. This review is restricted to symptomatic and supportive approaches since all attempts to establish a cure for the disease or modifying therapies have failed so far. Keywords: Neurodegeneration, clinical picture, early symptoms, therapy, treatment

  16. Ethical considerations of genetic presymptomatic testing for Huntington's disease.

    Science.gov (United States)

    Coustasse, Alberto; Pekar, Alicia; Sikula, Andrew; Lurie, Sue

    2009-01-01

    The aim of this literature review was to determine if there is adequate ethical justification for presymptomatic genetic testing on potential Huntington's disease patients. Huntington's disease is a neurological genetic disorder characterized by midlife onset which consists of cognitive, physical, and emotional deterioration. Although genetic testing has traditionally been guided by the principle of autonomy, severe psychological consequences such as depression, anxiety, survival guilt, and suicide have complicated the ethical issue of providing a presymptomatic yet definitive diagnosis for an incurable disease. An analysis of available articles yielded inconclusive findings, namely due to insufficient evidence, self-selection bias of test participants, or lack of a longitudinal design. Additional results indicated psychological distress is not solely associated with test result, but rather with individual characteristics including, but not limited to, psychological history, test motivation, level of preparation, social support, and age. In the interest of upholding the principles of autonomy, beneficence, nonmaleficence, and justice, it is recommended that medical professionals follow strict protocol, provide extensive counseling, and employ vigilance when assessing at-risk individuals for HD presymptomatic test eligibility to ensure psychological well-being.

  17. Comprehensive care in Huntington's disease: a physician's perspective.

    Science.gov (United States)

    Nance, Martha A

    2007-04-30

    Huntington's disease is a slowly progressive neurodegenerative disorder with wide-ranging effects on affected individuals and their families. Until a cure is found for the disease, patients and their families will continue to need care over years, even generations. The ideal care for HD is provided by a team, led by a physician, with input from rehabilitation therapists, nurses, psychologists, genetic counselors, social workers, and other health care providers. The goals of care are to maximize the quality of life at all points through the course of the disease, in part by anticipating problems that are likely to arise at the next stage of the illness. We describe below an approach to comprehensive care, and introduce the concept of the "Huntington disease molecule", in which the patient, in the center, is surrounded by a shell of immediate and extended family members, with bonds extended in multiple directions to provider who can give appropriate medical care, education, crisis management, research opportunities, address family issues, maximize function, and prepare for the future.

  18. Deep brain stimulation in Huntington's disease: assessment of potential targets.

    Science.gov (United States)

    Sharma, Mayur; Deogaonkar, Milind

    2015-05-01

    Huntington's disease (HD) is an autosomal-dominant neurodegenerative disorder that has very few effective therapeutic interventions. Since the disease has a defined neural circuitry abnormality, neuromodulation could be an option. Case reports, original research, and animal model studies were selected from the databases of Medline and PubMed. All related studies published up to July 2014 were included in this review. The following search terms were used: "Deep brain stimulation," "DBS," "thalamotomy," "pallidal stimulation," and "Huntington's Disease," "HD," "chorea," or "hyperkinetic movement disorders." This review examines potential nodes in the HD circuitry that could be modulated using deep brain stimulation (DBS) therapy. With rapid evolution of imaging and ability to reach difficult targets in the brain with refined DBS technology, some phenotypes of HD could potentially be treated with DBS in the near future. Further clinical studies are warranted to validate the efficacy of neuromodulation and to determine the most optimal target for HD. Copyright © 2014 Elsevier Ltd. All rights reserved.

  19. Functional impairment of precerebral arteries in Huntington disease.

    Science.gov (United States)

    Kobal, Jan; Cankar, Ksenija; Pretnar, Janja; Zaletel, Marjan; Kobal, Lucijan; Teran, Natasa; Melik, Ziva

    2017-01-15

    Cardiovascular pathology of Huntington disease (HD) appears to be complex; while microvascular dysfunction seems to appear early, deaths from cardiomyopathy and stroke might occur in the late phase of HD. Our study evaluated global risk factors for coronary heart disease (CHD), structure and function of precerebral arteries in 41 HD subjects and 41 matched controls. HD subjects were divided into groups by the United Huntington disease rating scale (presymptomatic-PHD, early-EHD, midstage-MHD and late-LHD). CHD risk factors assessment and Doppler examination of precerebral arteries were performed, including measurements of the carotid artery intima-media thickness (IMT), and parameters indicating local carotid artery distensibility (stiffness index β, pulse wave velocity, pressure strain elasticity module and carotid artery compliance). In the HD and controls we identified a comparable number of non-obstructive plaques (50% lumen narrowing) were found. There was significantly increased IMT in MHD. In PHD and EHD the parameters of arterial stiffness were significantly higher and the carotid artery compliance was significantly lower. Our results reveal functional vascular pathology in PHD, EHD, and MHD. Precerebral arteries dysfunction in HD therefore appears to be mostly functional and in agreement with recently described autonomic nervous system changes in HD. Copyright © 2016 Elsevier B.V. All rights reserved.

  20. Clinical and genetic data of Huntington disease in Moroccan patients.

    Science.gov (United States)

    Bouhouche, Ahmed; Regragui, Wafaa; Lamghari, Hind; Khaldi, Khadija; Birouk, Nazha; Lytim, Safaa; Bellamine, Soufiane; Kriouile, Yamna; Bouslam, Naima; Haddou, El Hachmia Ait Ben; Faris, Mustapha Alaoui; Benomar, Ali; Yahyaoui, Mohamed

    2015-12-01

    Huntington's disease (HD) occurs worldwide with prevalence varying from 0.1 to 10/100,000 depending of the ethnic origin. Since no data is available in the Maghreb population, the aim of this study is to describe clinical and genetic characteristics of Huntington patients of Moroccan origin. Clinical and genetics data of 21 consecutive patients recruited from 2009 to 2014 from the outpatient clinic of six medical centers were analyzed. Statistical analysis was performed using descriptive statistics. Twenty one patients from 17 families were diagnosed positive for the IT15 gene CAG expansion. Clinical symptoms were predominantly motor (19/21). Twelve patients had psychiatric and behavioral disorders, and 11 patients had cognitive disorders essentially of memory impairment. Analysis of genetic results showed that 5 patients had reduced penetrant (RP) alleles and 16 had fully penetrant (FP) alleles. The mean CAG repeat length in patients with RP alleles was 38.4 ± 0.54, and 45.37 ± 8.30 in FP alleles. The age of onset and the size of the CAG repeat length showed significant inverse correlation (p <0.001, r = -0.754). Clinical and genetic data of Moroccan patients are similar to those of Caucasian populations previously reported in the literature.

  1. Allegheny County Address Points

    Data.gov (United States)

    Allegheny County / City of Pittsburgh / Western PA Regional Data Center — This dataset contains address points which represent physical address locations assigned by the Allegheny County addressing authority. Data is updated by County...

  2. Allegheny County Air Quality

    Data.gov (United States)

    Allegheny County / City of Pittsburgh / Western PA Regional Data Center — Air quality data from Allegheny County Health Department monitors throughout the county. Air quality monitored data must be verified by qualified individuals before...

  3. Allegheny County Council Districts

    Data.gov (United States)

    Allegheny County / City of Pittsburgh / Western PA Regional Data Center — This dataset portrays the boundaries of the County Council Districts in Allegheny County. The dataset is based on municipal boundaries and City of Pittsburgh ward...

  4. Allegheny County Municipal Boundaries

    Data.gov (United States)

    Allegheny County / City of Pittsburgh / Western PA Regional Data Center — This dataset demarcates the municipal boundaries in Allegheny County. Data was created to portray the boundaries of the 130 Municipalities in Allegheny County the...

  5. Allegheny County Address Points

    Data.gov (United States)

    Allegheny County / City of Pittsburgh / Western PA Regional Data Center — This dataset contains address points which represent physical address locations assigned by the Allegheny County addressing authority. Data is updated by County...

  6. Allegheny County Council Districts

    Data.gov (United States)

    Allegheny County / City of Pittsburgh / Western PA Regional Data Center — This dataset portrays the boundaries of the County Council Districts in Allegheny County. The dataset is based on municipal boundaries and City of Pittsburgh ward...

  7. Allegheny County Municipal Boundaries

    Data.gov (United States)

    Allegheny County / City of Pittsburgh / Western PA Regional Data Center — This dataset demarcates the municipal boundaries in Allegheny County. Data was created to portray the boundaries of the 130 Municipalities in Allegheny County the...

  8. An Evaluation of Market Characteristics of Indiana Farmers' Markets

    OpenAIRE

    Hofmann, Christa; Dennis, Jennifer H.; Marshall, Maria I

    2008-01-01

    Nationally, the number of operating farmers' markets has increased 111% in the past ten years from 1,755 markets to 3,706 from 1994 to 2004 (AMS, 2006). Indiana's farmers' markets has increased at double the rate in the same time frame. An internet and mail census was sent to market masters to assess operational procedures and factors that influence customer and vendor participation in the market. A two-stage least squares model was estimated for the vendor and customer model. In Equation 1.1...

  9. Mercury in Indiana watersheds: retrospective for 2001-2006

    Science.gov (United States)

    Risch, Martin R.; Baker, Nancy T.; Fowler, Kathleen K.; Egler, Amanda L.; Lampe, David C.

    2010-01-01

    Information about total mercury and methylmercury concentrations in water samples and mercury concentrations in fish-tissue samples was summarized for 26 watersheds in Indiana that drain most of the land area of the State. Mercury levels were interpreted with information on streamflow, atmospheric mercury deposition, mercury emissions to the atmosphere, mercury in wastewater, and landscape characteristics. Unfiltered total mercury concentrations in 411 water samples from streams in the 26 watersheds had a median of 2.32 nanograms per liter (ng/L) and a maximum of 28.2 ng/L. When these concentrations were compared to Indiana water-quality criteria for mercury, 5.4 percent exceeded the 12-ng/L chronic-aquatic criterion, 59 percent exceeded the 1.8-ng/L Great Lakes human-health criterion, and 72.5 percent exceeded the 1.3-ng/L Great Lakes wildlife criterion. Mercury concentrations in water were related to streamflow, and the highest mercury concentrations were associated with the highest streamflows. On average, 67 percent of total mercury in streams was in a particulate form, and particulate mercury concentrations were significantly lower downstream from dams than at monitoring stations not affected by dams. Methylmercury is the organic fraction of total mercury and is the form of mercury that accumulates and magnifies in food chains. It is made from inorganic mercury by natural processes under specific conditions. Unfiltered methylmercury concentrations in 411 water samples had a median of 0.10 ng/L and a maximum of 0.66 ng/L. Methylmercury was a median 3.7 percent and maximum 64.8 percent of the total mercury in 252 samples for which methylmercury was reported. The percentages of methylmercury in water samples were significantly higher downstream from dams than at other monitoring stations. Nearly all of the total mercury detected in fish tissue was assumed to be methylmercury. Fish-tissue samples from the 26 watersheds had wet-weight mercury concentrations that

  10. Sunrise coal, an innovative New Indiana player continues to grow

    Energy Technology Data Exchange (ETDEWEB)

    Buchsbaum, L.

    2009-07-15

    Sunrise Coal LLC's Carliste (Indiana) underground mine began development in 2006. Today, the room and pillar operation has grown to a 3 million tpy four unit continuous miner mine. Its coal has low (0.06%) chlorine level and is now being purchased to blend down high chlorine in Illinois Basin coal. The article describes the mining operation and equipment traces the growth of the company, founded in the 1970s by Row and Steve Laswell, emphasizing its focus on employee safety. 5 photos.

  11. Digital bedrock geologic map of parts of the Huntington, Richmond, Bolton and Waterbury quadrangles, Vermont

    Data.gov (United States)

    Vermont Center for Geographic Information — Digital Data from VG95-9A Thompson, PJ�and Thompson, TB, 1995, Digital bedrock geologic map of parts of the Huntington, Richmond, Bolton and Waterbury quadrangles,...

  12. Recent Trends in Detection of Huntingtin and Preclinical Models of Huntington's Disease.

    Science.gov (United States)

    Mantha, Neelima; Das, Nandita G; Das, Sudip K

    2014-01-01

    Huntington's disease is a genetically inherited neurodegenerative disease that is characterized by neuronal cell death in the brain. Molecular biology techniques to detect and quantify huntingtin protein in biological samples involve fluorescence imaging, western blotting, and PCR. Modified cell lines are widely used as models for Huntington's disease for preclinical screening of drugs to study their ability to suppress the expression of huntingtin. Although worm and fly species have been experimented on as models for Huntington's disease, the most successful animal models have been reported to be primates. This review critically analyses the molecular biology techniques for detection and quantitation of huntingtin and evaluates the various animal species for use as models for Huntington's disease.

  13. The Current Status of Neural Grafting in the Treatment of Huntington's Disease. A Review

    National Research Council Canada - National Science Library

    Wijeyekoon, Ruwani; Barker, Roger A

    2011-01-01

    Huntington's disease (HD) is a devastating, fatal, autosomal dominant condition in which the abnormal gene codes for a mutant form of huntingtin that causes widespread neuronal dysfunction and death...

  14. Hypothalamic Alterations in Huntington's Disease Patients : Comparison with Genetic Rodent Models

    NARCIS (Netherlands)

    Van Wamelen, D.J.; Aziz, N A; Roos, R A C; Swaab, D F

    2014-01-01

    Unintended weight loss, sleep and circadian disturbances and autonomic dysfunction are prevalent features of Huntington's disease (HD), an autosomal dominantly inherited neurodegenerative disorder caused by an expanded CAG repeat sequence in the HTT gene. These features form a substantial contributi

  15. 77 FR 51064 - Huntington Foam LLC, Fort Smith, AR; Notice of Affirmative Determination Regarding Application...

    Science.gov (United States)

    2012-08-23

    ... From the Federal Register Online via the Government Publishing Office DEPARTMENT OF LABOR Employment and Training Administration Huntington Foam LLC, Fort Smith, AR; Notice of Affirmative Determination Regarding Application for Reconsideration By application dated May 21, 2012, the State...

  16. The role of tau in the pathological process and clinical expression of Huntington's disease

    DEFF Research Database (Denmark)

    Vuono, Romina; Winder-Rhodes, Sophie; de Silva, Rohan

    2015-01-01

    -mortem brain samples from patients with Huntington's disease (n = 16) compared to cases with a known tauopathy and healthy controls. Next, we undertook a genotype-phenotype analysis of a large cohort of patients with Huntington's disease (n = 960) with a particular focus on cognitive decline. We report...... not only on the tau pathology in the Huntington's disease brain but also the association between genetic variation in tau gene and the clinical expression and progression of the disease. We found extensive pathological inclusions containing abnormally phosphorylated tau protein that co-localized in some...... instances with mutant HTT. We confirmed this related to the disease process rather than age, by showing it is also present in two patients with young-onset Huntington's disease (26 and 40 years old at death). In addition we demonstrate that tau oligomers (suggested to be the most likely neurotoxic tau...

  17. Dynamics of the connectome in Huntington's disease : A longitudinal diffusion MRI study

    NARCIS (Netherlands)

    Odish, Omar F F; Caeyenberghs, Karen; Hosseini, Hadi; Van Den Bogaard, Simon J A; Roos, Raymund A C; Leemans, A

    2015-01-01

    Abstract Objectives To longitudinally investigate the connectome in different stages of Huntington's disease (HD) by applying graph theoretical analysis to diffusion MRI data. Experimental design We constructed weighted structural networks and calculated their topological properties. Twenty-two prem

  18. Evaluation of tetrathiomolybdate in the R6/2 model of Huntington disease.

    Science.gov (United States)

    Tallaksen-Greene, Sara J; Janiszewska, Anita; Benton, Kasha; Hou, Guoqing; Dick, Robert; Brewer, George J; Albin, Roger L

    2009-03-06

    Huntington disease is an uncommon autosomal dominant neurodegenerative disorder caused by expanded polyglutamine repeats in the huntingtin protein. The proximate mechanisms responsible for neurodegeneration are unknown. Copper ions may play a role in Huntington disease by promoting oligomerization of expanded polyglutamine repeat protein fragments. Ammonium tetrathiomolybdate is a copper complexing agent with demonstrated tolerability and efficacy in another neurodegenerative disorder, Wilson disease. We evaluated ammonium tetrathiomolybdate in the R6/2 transgenic mouse model of Huntington disease. Ammonium tetrathiomolybdate treatment delayed the onset of motor dysfunction in R6/2 mice. There was a trend towards reduced striatal degeneration, suggesting a neuroprotective effect of ammonium tetrathiomolybdate in this model. Given its known tolerability in humans with neurodegeneration, ammonium tetrathiomolybdate could be considered as a candidate for clinical trials in Huntington disease.

  19. The role of tau in the pathological process and clinical expression of Huntington's disease.

    Science.gov (United States)

    Vuono, Romina; Winder-Rhodes, Sophie; de Silva, Rohan; Cisbani, Giulia; Drouin-Ouellet, Janelle; Spillantini, Maria G; Cicchetti, Francesca; Barker, Roger A

    2015-07-01

    Huntington's disease is a neurodegenerative disorder caused by an abnormal CAG repeat expansion within exon 1 of the huntingtin gene HTT. While several genetic modifiers, distinct from the Huntington's disease locus itself, have been identified as being linked to the clinical expression and progression of Huntington's disease, the exact molecular mechanisms driving its pathogenic cascade and clinical features, especially the dementia, are not fully understood. Recently the microtubule associated protein tau, MAPT, which is associated with several neurodegenerative disorders, has been implicated in Huntington's disease. We explored this association in more detail at the neuropathological, genetic and clinical level. We first investigated tau pathology by looking for the presence of hyperphosphorylated tau aggregates, co-localization of tau with mutant HTT and its oligomeric intermediates in post-mortem brain samples from patients with Huntington's disease (n = 16) compared to cases with a known tauopathy and healthy controls. Next, we undertook a genotype-phenotype analysis of a large cohort of patients with Huntington's disease (n = 960) with a particular focus on cognitive decline. We report not only on the tau pathology in the Huntington's disease brain but also the association between genetic variation in tau gene and the clinical expression and progression of the disease. We found extensive pathological inclusions containing abnormally phosphorylated tau protein that co-localized in some instances with mutant HTT. We confirmed this related to the disease process rather than age, by showing it is also present in two patients with young-onset Huntington's disease (26 and 40 years old at death). In addition we demonstrate that tau oligomers (suggested to be the most likely neurotoxic tau entity) are present in the Huntington's disease brains. Finally we highlight the clinical significance of this pathology by demonstrating that the MAPT haplotypes affect the rate

  20. Increased brain tissue sodium concentration in Huntington's Disease - a sodium imaging study at 4 T.

    Science.gov (United States)

    Reetz, Kathrin; Romanzetti, Sandro; Dogan, Imis; Saß, Christian; Werner, Cornelius J; Schiefer, Johannes; Schulz, Jörg B; Shah, N Jon

    2012-10-15

    The neuropathological hallmark of the autosomal dominantly inherited, neurodegenerative disorder Huntington's disease is progressive striatal loss starting several years prior to symptom manifestation. Magnetic resonance (MR) imaging has been widely used to detect altered structure in premanifest and early Huntington's disease. Given that neurodegeneration is likely preceded by substantial neuronal dysfunction, we used in vivo sodium MR imaging, which has been shown to be sensitive to cell death and viability, to investigate cellular and metabolic integrity of Huntington's disease brain tissue. We studied a total of thirteen healthy controls and thirteen Huntington's disease gene carriers (11 manifest and 2 premanifest). The manifest Huntington's disease group was subdivided into stages 1 and 2 according to their Total Functional Capacity scores. Clinical total motor and cognitive scores, as well as calibrated sodium and T1-weighted MR images were obtained with a 4 T Siemens MR scanner. Sodium images were acquired by means of a constant time imaging technique with an ultra-short "echo time". T1-weighted MR images were further analysed with voxel-based morphometry. The absolute total sodium concentration and grey matter values were measured in several Huntington's disease-specific and also non-specific areas. Statistical analysis of variance and Pearson correlation were applied. In Huntington's disease subjects, we found an increase of total sodium concentration of the entire brain compared to controls. Increased total sodium concentration values were found in structurally affected, but also in some non-affected, regions. The highest total sodium concentration values were found in the bilateral caudate, which was associated with caudate grey matter atrophy and CAG repeat length. In all Huntington's disease subjects we further found a profound increase of total sodium concentration in the putamen, pallidum, thalamus, hippocampus, insula, precuneus and occipital

  1. [The life as a caregiver of a person affected by Chorea Huntington: multiple case study].

    Science.gov (United States)

    Winkler, Evi; Ausserhofer, Dietmar; Mantovan, Franco

    2012-10-01

    Chorea Huntington is an autosomal dominantly inherited, neurodegenerative brain disorder that leads to involuntary hyperkinesia, psychotic symptoms and dementia. The illness not only changes the life of the person itself but also the world of the caregivers. The challenges in the care of a person which is affected by Chorea Huntington have an effect on the daily living as an assemblage of natural and social conditions. a multiple case study was conducted. It included semi-structured interviews with three caregivers of people with Chorea Huntington in South Tyrol. The qualitative data was analyzed using the qualitative structured analysis of Mayring (2007). The objective of this study was to describe the phenomenon of change of life from family members that care people affected by Chorea Huntington in a specific cultural setting (South Tyrol, Italy). The caregivers reported that the diagnosis of Chorea Huntington leads to negative changes in "relationship and family". Particularly, frustration, aggression, impatience and apathy were perceived as stressful. At the same time they highlight the positive changes through home care. They report that the relationship became more intimate and integral and it was characterized by more cohesion. Family caregivers get valuable support from the home care service, however, they complain that there is no facility in South Tyrol, which is specialized to care people with Chorea Huntington. Therefore, the caregivers have to "give up a lot" and don't have any personal desires, dreams and expectations for the future. The caregivers have learned independently to deal with their changed life step by step, and to see also the positive effects of the caring role. The life of family caregivers of a person which is affected by Chorea Huntington is characterized by abandonment. A continuous and professional care would be important for the affected and his caregiver. A continuous and professional care is important for both, addressing the

  2. A double blind trial of sulpiride in Huntington's disease and tardive dyskinesia.

    OpenAIRE

    Quinn, N.; Marsden, C. D.

    1984-01-01

    Eleven patients with Huntington's disease and nine patients with tardive dyskinesia participated in a randomised double-blind crossover trial of sulpiride (as sole antidopaminergic therapy) versus placebo. Although functional improvement was not seen in Huntington's disease patients, sulpiride reduced movement count and total dyskinesia score in both conditions. Sulpiride differs pharmacologically in several respects from conventional neuroleptics, and has not been convincingly shown to cause...

  3. Economic Impacts from Indiana's First 1,000 Megawatts of Wind Power

    Energy Technology Data Exchange (ETDEWEB)

    Tegen, S.; Keyser, D.; Flores-Espino, F.; Hauser, R.

    2014-08-01

    The magnitude of Indiana's available wind resource indicates that the development of wind power infrastructure has the potential to support millions of dollars of economic activity in the state. The Jobs and Economic Development Impact (JEDI) models, developed by the National Renewable Energy Laboratory, are tools used to estimate some of the economic impacts of energy projects at the state level. JEDI calculates results in the form of jobs, earnings, and economic output in three categories: project development and onsite labor, local revenue and supply chain, and induced impacts. According to this analysis, the first 1,000 MW of wind power development in Indiana (projects built between 2008 and 2011): supported employment totaling more than 4,400 full-time-equivalent jobs in Indiana during the construction periods; supports approximately 260 ongoing Indiana jobs; supported nearly $570 million in economic activity for Indiana during the construction periods; supported and continues to support nearly $40 million in annual Indiana economic activity during the operating periods; generates more than $8 million in annual property taxes; generates nearly $4 million annually in income for Indiana landowners who lease their land for wind energy projects.

  4. It wasn't Witchcraft--It was Huntington Disease!

    Science.gov (United States)

    Penaranda, Eribeth; Garcia, Angel; Montgomery, Lisa

    2011-01-01

    Huntington disease (HD) is an autosomal-dominant, incurable, progressive disorder that manifests with chorea and behavioral and cognitive impairment. The disease usually occurs during the fourth or fifth decade of life; however, it may present at any age. Clinical suspicion is confirmed by genetic testing. Death occurs, on average, 15 to 20 years after the onset of symptoms. Here we report about a Hispanic woman and her family who were affected by the disease; this case illustrates the role of cultural values and beliefs in the decision-making process, as well as the importance of the physician's cultural competency in fostering a trusting relationship that may lessen the burden of catastrophic diseases on individuals, families, and society at-large.

  5. Huntington's disease impairs recognition of angry and instrumental body language.

    Science.gov (United States)

    de Gelder, Beatrice; Van den Stock, Jan; Balaguer, Ruth de Diego; Bachoud-Lévi, Anne-Catherine

    2008-01-15

    Patients with Huntington's disease (HD) exhibit motor impairments as well as cognitive and emotional deficits. So far impairments in the ability to recognize emotional stimuli have mostly been investigated by using facial expressions and emotional voices. Other important emotional signals are provided by the whole body. To investigate the impact of motor deficits on body recognition and the relation between motor disorders and emotion perception deficits, we tested recognition of emotional body language (instrumental, angry, fearful and sad) in 19 HD patients and their matched controls with a nonverbal whole body expression matching task. Results indicate that HD patients are impaired in recognizing both instrumental and angry whole body postures. Furthermore, the body language perception deficits are correlated with measures of motor deficit. Taken together the results suggest a close relationship between emotion recognition (specifically anger) and motor abilities.

  6. Rapid eye movement sleep disturbances in Huntington disease

    DEFF Research Database (Denmark)

    Arnulf, I.; Nielsen, J.; Lohmann, E.

    2008-01-01

    with very mild HD and worsened with disease severity. In contrast to narcoleptic patients, HD patients had no cataplexy, hypnagogic hallucinations, or sleep paralysis. Four HD patients had abnormally low (sleep latencies, but none had multiple sleep-onset REM periods. Conclusions......Background: Sleep disorders including insomnia, movements during sleep, and daytime sleepiness are common but poorly studied in Huntington disease (HD). Objective: To evaluate the HD sleep-wake phenotype (including abnormal motor activity during sleep) in patients with various HD stages...... interview, nighttime video and sleep monitoring, and daytime multiple sleep latency tests. Their results were compared with those of patients with narcolepsy and control patients. Results: The HD patients had frequent insomnia, earlier sleep onset, lower sleep efficiency, increased stage I sleep, delayed...

  7. Nucleic Acid-Based Therapy Approaches for Huntington's Disease

    Directory of Open Access Journals (Sweden)

    Tatyana Vagner

    2012-01-01

    Full Text Available Huntington's disease (HD is caused by a dominant mutation that results in an unstable expansion of a CAG repeat in the huntingtin gene leading to a toxic gain of function in huntingtin protein which causes massive neurodegeneration mainly in the striatum and clinical symptoms associated with the disease. Since the mutation has multiple effects in the cell and the precise mechanism of the disease remains to be elucidated, gene therapy approaches have been developed that intervene in different aspects of the condition. These approaches include increasing expression of growth factors, decreasing levels of mutant huntingtin, and restoring cell metabolism and transcriptional balance. The aim of this paper is to outline the nucleic acid-based therapeutic strategies that have been tested to date.

  8. Unravelling and Exploiting Astrocyte Dysfunction in Huntington's Disease.

    Science.gov (United States)

    Khakh, Baljit S; Beaumont, Vahri; Cachope, Roger; Munoz-Sanjuan, Ignacio; Goldman, Steven A; Grantyn, Rosemarie

    2017-07-01

    Astrocytes are abundant within mature neural circuits and are involved in brain disorders. Here, we summarize our current understanding of astrocytes and Huntington's disease (HD), with a focus on correlative and causative dysfunctions of ion homeostasis, calcium signaling, and neurotransmitter clearance, as well as on the use of transplanted astrocytes to produce therapeutic benefit in mouse models of HD. Overall, the data suggest that astrocyte dysfunction is an important contributor to the onset and progression of some HD symptoms in mice. Additional exploration of astrocytes in HD mouse models and humans is needed and may provide new therapeutic opportunities to explore in conjunction with neuronal rescue and repair strategies. Copyright © 2017 Elsevier Ltd. All rights reserved.

  9. Genetic counseling and testing for Huntington's disease: A historical review.

    Science.gov (United States)

    Nance, Martha A

    2017-01-01

    This manuscript describes the ways in which genetic counseling has evolved since John Pearson and Sheldon Reed first promoted "a genetic education" in the 1950s as a voluntary, non-directive clinical tool for permitting individual decision making. It reviews how the emergence of Huntington's disease (HD) registries and patient support organizations, genetic testing, and the discovery of a disease-causing CAG repeat expansion changed the contours of genetic counseling for families with HD. It also reviews the guidelines, outcomes, ethical and laboratory challenges, and uptake of predictive, prenatal, and preimplantation testing, and it casts a vision for how clinicians can better make use of genetic counseling to reach a broader pool of families that may be affected by HD and to ensure that genetic counseling is associated with the best levels of care. © 2016 Wiley Periodicals, Inc.

  10. Modern Genome Editing Technologies in Huntington's Disease Research.

    Science.gov (United States)

    Malankhanova, Tuyana B; Malakhova, Anastasia A; Medvedev, Sergey P; Zakian, Suren M

    2017-01-01

    The development of new revolutionary technologies for directed gene editing has made it possible to thoroughly model and study NgAgo human diseases at the cellular and molecular levels. Gene editing tools like ZFN, TALEN, CRISPR-based systems, NgAgo and SGN can introduce different modifications. In gene sequences and regulate gene expression in different types of cells including induced pluripotent stem cells (iPSCs). These tools can be successfully used for Huntington's disease (HD) modeling, for example, to generate isogenic cell lines bearing different numbers of CAG repeats or to correct the mutation causing the disease. This review presents common genome editing technologies and summarizes the progress made in using them in HD and other hereditary diseases. Furthermore, we will discuss prospects and limitations of genome editing in understanding HD pathology.

  11. Striatal Vulnerability in Huntington's Disease: Neuroprotection Versus Neurotoxicity.

    Science.gov (United States)

    Morigaki, Ryoma; Goto, Satoshi

    2017-06-07

    Huntington's disease (HD) is an autosomal dominant neurodegenerative disease caused by the expansion of a CAG trinucleotide repeat encoding an abnormally long polyglutamine tract (PolyQ) in the huntingtin (Htt) protein. In HD, striking neuropathological changes occur in the striatum, including loss of medium spiny neurons and parvalbumin-expressing interneurons accompanied by neurodegeneration of the striosome and matrix compartments, leading to progressive impairment of reasoning, walking and speaking abilities. The precise cause of striatal pathology in HD is still unknown; however, accumulating clinical and experimental evidence suggests multiple plausible pathophysiological mechanisms underlying striatal neurodegeneration in HD. Here, we review and discuss the characteristic neurodegenerative patterns observed in the striatum of HD patients and consider the role of various huntingtin-related and striatum-enriched proteins in neurotoxicity and neuroprotection.

  12. Genetic Mouse Models of Huntington's Disease: Focus on Electrophysiological Mechanisms

    Directory of Open Access Journals (Sweden)

    Carlos Cepeda

    2010-03-01

    Full Text Available The discovery of the HD (Huntington's disease gene in 1993 led to the creation of genetic mouse models of the disease and opened the doors for mechanistic studies. In particular, the early changes and progression of the disease could be followed and examined systematically. The present review focuses on the contribution of these genetic mouse models to the understanding of functional changes in neurons as the HD phenotype progresses, and concentrates on two brain areas: the striatum, the site of most conspicuous pathology in HD, and the cortex, a site that is becoming increasingly important in understanding the widespread behavioural abnormalities. Mounting evidence points to synaptic abnormalities in communication between the cortex and striatum and cell-cell interactions as major determinants of HD symptoms, even in the absence of severe neuronal degeneration and death.

  13. High resolution impedance manometric findings in dysphagia of Huntington's disease

    Institute of Scientific and Technical Information of China (English)

    Tae Hee Lee; Joon Seong Lee; Wan Jung Kim

    2012-01-01

    Conventional manometry presents significant challenges,espedally in assessment of pharyngeal swallowing,because of the asymmetry and deglutitive movements of oropharyngeal structures.It only provides information about intraluminal pressure and thus it is difficult to study functional details of esophageal motility disorders.New technology of solid high resolution impedance manometry (HRIM),with 32 pressure sensors and 6 impedance sensors,is likely to provide better assessment of pharyngeal swallowing as well as more information about esophageal motility disorders.However,the clinical usefulness of application of HRIM in patients with oropharyngeal dysphagia or esophageal dysphagia is not known.We experienced a case of Huntington's disease presenting with both oropharyngeal and esophageal dysphagia,in which HRIM revealed the mechanism of oropharyngeal dysphagia and provided comprehensive information about esophageal dysphagia.

  14. Observation of the Wigner-Huntington transition to metallic hydrogen

    Science.gov (United States)

    Dias, Ranga P.; Silvera, Isaac F.

    2017-02-01

    Producing metallic hydrogen has been a great challenge in condensed matter physics. Metallic hydrogen may be a room-temperature superconductor and metastable when the pressure is released and could have an important impact on energy and rocketry. We have studied solid molecular hydrogen under pressure at low temperatures. At a pressure of 495 gigapascals, hydrogen becomes metallic, with reflectivity as high as 0.91. We fit the reflectance using a Drude free-electron model to determine the plasma frequency of 32.5 ± 2.1 electron volts at a temperature of 5.5 kelvin, with a corresponding electron carrier density of 7.7 ± 1.1 × 1023 particles per cubic centimeter, which is consistent with theoretical estimates of the atomic density. The properties are those of an atomic metal. We have produced the Wigner-Huntington dissociative transition to atomic metallic hydrogen in the laboratory.

  15. Westphal variant Huntington disease and refractory catatonia: a case report.

    Science.gov (United States)

    Merida-Puga, Jorge; Ramirez-Bermudez, Jesus; Aguilar-Venegas, Luis Carlos; Fricchione, Gregory L; Espinola-Nadurille, Mariana

    2011-12-01

    A young woman with Westphal variant (juvenile) Huntington disease (HD) also developed catatonia. Catatonia is an underdiagnosed psychomotor syndrome often associated with neurological and psychiatric disorders, but it has rarely been documented in patients with HD. Catatonia usually responds to standard treatment with benzodiazepines and electroconvulsive therapy; however, this patient's catatonic syndrome did not improve until we augmented the standard treatment with amantadine and levodopa. The underlying pathophysiology and a neurochemical hypothesis of HD and catatonia can explain their comorbidity and the refractoriness of catatonia to treatment. Both conditions are linked to dysregulation of neurotransmitters in the striatocortical and corticocortical pathways. This understanding may serve as a guide for the use of nonstandard treatments. Our evidence also suggests that electroconvulsive therapy can be useful and safe in the treatment of HD.

  16. Autophagy in Huntington disease and huntingtin in autophagy.

    Science.gov (United States)

    Martin, Dale D O; Ladha, Safia; Ehrnhoefer, Dagmar E; Hayden, Michael R

    2015-01-01

    Autophagy is an important biological process that is essential for the removal of damaged organelles and toxic or aggregated proteins by delivering them to the lysosome for degradation. Consequently, autophagy has become a primary target for the treatment of neurodegenerative diseases that involve aggregating proteins. In Huntington disease (HD), an expansion of the polyglutamine (polyQ) tract in the N-terminus of the huntingtin (HTT) protein leads to protein aggregation. However, HD is unique among the neurodegenerative proteinopathies in that autophagy is not only dysfunctional but wild type (wt) HTT also appears to play several roles in regulating the dynamics of autophagy. Herein, we attempt to integrate the recently described novel roles of wtHTT and altered autophagy in HD. Copyright © 2014 Elsevier Ltd. All rights reserved.

  17. Diagnóstico molecular de la enfermedad de Huntington en Costa Rica Molecular diagnosis of Huntington´s disease in Costa Rica

    OpenAIRE

    Melissa Vásquez-Cerdas; Fernando Morales-Montero; Húbert Fernández-Morales; Gerardo el Valle-Carazo; Jaime Fornaguera-Trías; Patricia Cuenca-Berger

    2008-01-01

    Artículo científico -- Universidad de Costa Rica. Instituto de Investigaciones en Salud, 2008 Justificación y objetivo. Este estudio representa un esfuerzo para establecer por primera vez en Costa Rica el diagnóstico molecular de la enfermedad de Huntington; esto favorecerá un mejor manejo clínico de los pacientes y podrá ser traducido en un incremento de la calidad de vida de las familias. Se pretende determinar el número de repeticiones CAG en personas con la enfermedad de Huntington y f...

  18. Discrepancies in reporting the CAG repeat lengths for Huntington's disease.

    Science.gov (United States)

    Quarrell, Oliver W; Handley, Olivia; O'Donovan, Kirsty; Dumoulin, Christine; Ramos-Arroyo, Maria; Biunno, Ida; Bauer, Peter; Kline, Margaret; Landwehrmeyer, G Bernhard

    2012-01-01

    Huntington's disease results from a CAG repeat expansion within the Huntingtin gene; this is measured routinely in diagnostic laboratories. The European Huntington's Disease Network REGISTRY project centrally measures CAG repeat lengths on fresh samples; these were compared with the original results from 121 laboratories across 15 countries. We report on 1326 duplicate results; a discrepancy in reporting the upper allele occurred in 51% of cases, this reduced to 13.3% and 9.7% when we applied acceptable measurement errors proposed by the American College of Medical Genetics and the Draft European Best Practice Guidelines, respectively. Duplicate results were available for 1250 lower alleles; discrepancies occurred in 40% of cases. Clinically significant discrepancies occurred in 4.0% of cases with a potential unexplained misdiagnosis rate of 0.3%. There was considerable variation in the discrepancy rate among 10 of the countries participating in this study. Out of 1326 samples, 348 were re-analysed by an accredited diagnostic laboratory, based in Germany, with concordance rates of 93% and 94% for the upper and lower alleles, respectively. This became 100% if the acceptable measurement errors were applied. The central laboratory correctly reported allele sizes for six standard reference samples, blind to the known result. Our study differs from external quality assessment (EQA) schemes in that these are duplicate results obtained from a large sample of patients across the whole diagnostic range. We strongly recommend that laboratories state an error rate for their measurement on the report, participate in EQA schemes and use reference materials regularly to adjust their own internal standards.

  19. Orphan drugs in development for Huntington's disease: challenges and progress

    Directory of Open Access Journals (Sweden)

    Burgunder JM

    2015-02-01

    advanced strategies to develop novel treatments in Huntington's disease are examined. Keywords: Huntington's disease, symptomatic treatment, disease-modifying therapy

  20. Altered brain mechanisms of emotion processing in pre-manifest Huntington's disease.

    Science.gov (United States)

    Novak, Marianne J U; Warren, Jason D; Henley, Susie M D; Draganski, Bogdan; Frackowiak, Richard S; Tabrizi, Sarah J

    2012-04-01

    Huntington's disease is an inherited neurodegenerative disease that causes motor, cognitive and psychiatric impairment, including an early decline in ability to recognize emotional states in others. The pathophysiology underlying the earliest manifestations of the disease is not fully understood; the objective of our study was to clarify this. We used functional magnetic resonance imaging to investigate changes in brain mechanisms of emotion recognition in pre-manifest carriers of the abnormal Huntington's disease gene (subjects with pre-manifest Huntington's disease): 16 subjects with pre-manifest Huntington's disease and 14 control subjects underwent 1.5 tesla magnetic resonance scanning while viewing pictures of facial expressions from the Ekman and Friesen series. Disgust, anger and happiness were chosen as emotions of interest. Disgust is the emotion in which recognition deficits have most commonly been detected in Huntington's disease; anger is the emotion in which impaired recognition was detected in the largest behavioural study of emotion recognition in pre-manifest Huntington's disease to date; and happiness is a positive emotion to contrast with disgust and anger. Ekman facial expressions were also used to quantify emotion recognition accuracy outside the scanner and structural magnetic resonance imaging with voxel-based morphometry was used to assess the relationship between emotion recognition accuracy and regional grey matter volume. Emotion processing in pre-manifest Huntington's disease was associated with reduced neural activity for all three emotions in partially separable functional networks. Furthermore, the Huntington's disease-associated modulation of disgust and happiness processing was negatively correlated with genetic markers of pre-manifest disease progression in distributed, largely extrastriatal networks. The modulated disgust network included insulae, cingulate cortices, pre- and postcentral gyri, precunei, cunei, bilateral putamena