macular dystrophy 2-like: Topics by WorldWideScience.org

A YAC contig encompassing the recessive Stargardt disease gene (STGD) on chromosome 1p

Stargardt`s disease (MIM 248200), originally described in 1909, is an autosomal recessive condition of childhood, characterized by a sudden and bilateral loss of central vision. Typically, it has an early onset (7 to 12 years), a rapidly progressive course and a poor final outcome. The central area of the retina (macula) displays pigmentary changes in a ring form with depigmentation and atrophy of the retinal pigmentary epithelium (RPE). Perimacular yellowish spots, termed fundus flavimaculatus, are observed in a high percentage of patients. We have recently reported the genetic mapping of Stargardt`s disease to chromosome 1p13. On the other hand, considering that fundus flavimaculatus (MIM 230100) is another form of fleck fundus disease, with a Stargardt-like retinal aspect but with a late-onset and a more progressive course, we decided to test the hypothesis of allelism between typical Stargardt`s disease and late-onset autosomal recessive fundus flavimaculatus. Significant pairwise ...

1994-09-01

Macular Pigment Density Evolution in Unilateral Wet AMD Versus Non AMD Patients With or Without Lutein and Zeaxanthine Supplementation

Stargardt disease (STGD) and fundus flavimaculatus are infrequent autosomal recessive conditions characterized by a juvenile macular dystrophy and variable degrees of peripheral retinal changes. Linkage analysis performed in 47 STGD/fundus flavimaculatus families demonstrated significant linkage to 13 polymorphic DNA markers on chromosome 1p. The maximum combined two-point lod score was 32.7 (maximum recombination fraction [{theta}{sub max}] = .006) with the polymorphic marker D1S188. Our data demonstrate that STGD and fundus flavimaculatus are the same disorder clinically and genetically and provide further evidence for genetic homogeneity of this phenotype. Analysis of recombination on disease chromosomes placed the STGD gene within a 4-cM interval between markers D1S435 and D1S236. A physical map was constructed of a YAC contig flanking STGD, from markers D1S500 to D1S495, and includes the critical interval delineated by historical ...

1995-12-01

Abnormalities of dystrophin, the sarcoglycans, and laminin alpha2 in the muscular dystrophies.

Exudative Age-related Macular Degeneration

2011-08-03

Genetics and molecular pathology of Stargardt-like macular degeneration

Abnormalities of dystrophin, the sarcoglycans, and laminin alpha2 are responsible for a subset of the muscular dystrophies. In this study we aim to characterise the nature and frequency of abnormalities...Full Text Available

1998-05-01

If I Had - A Family History of Muscular Dystrophy

Stargardt-like macular degeneration (STGD3) is an early onset, autosomal dominant macular degeneration. STGD3 is characterized by a progressive pathology, the loss of central vision, atrophy...Full Text Available

2010-05-01

Medline Plus

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Optical Coherence Tomography Findings in Idiopathic Macular Holes

Genetic and physical location of the gene for Stargardt`s disease and further evidence for genetic homogeneity

Purpose. To describe the characteristics of idiopathic macular holes (MH) on optical coherence tomography (OCT) and correlate OCT with clinical assessment. Design....Full Text Available

2011-01-01

Schindler disease: the molecular lesion in the alpha-N-acetylgalactosaminidase gene that causes an infantile neuroaxonal dystrophy.

Stargardt`s disease is inherited as an autosomal recessive condition characterized by a juvenile macular dystrophy. Genetic linkage analysis recently assigned the disease locus to chromosome 1p21-p13 with the best estimate for location of the gene near the locus D1S435. We performed linkage analysis in 34 North American families and 2 inbred families from the Kingdom of Saudi Arabia with 12 highly polymorphic markers on chromosome 1p flanking D1S435 between D1S207 and D1S223 and report significant linkage for all 12 markers with no evidence for genetic heterogeneity. Two-point linkage analysis demonstrated the Stargardt`s disease locus and D1S435 are linked with a maximum lod score of 17.17 at a recombination fraction of 1%. The markers UT851, D1S188, D1S424, UT2069, and D1S236 also demonstrated recombination fractions of 1% or less with two-point lod scores of 15.86, 21.93, 16.41, 20.36, and 17.37, respectively. To characterize this region ...

1994-09-01

Mitigation of muscular dystrophy in mice by SERCA overexpression in skeletal muscle

Schindler disease is a recently recognized infantile neuroaxonal dystrophy resulting from the deficient activity of the lysosomal hydrolase, alpha-N-acetylgalctosaminidase (alpha-GalNAc). The recent...Full Text Available

1990-11-01

In vivo confocal microscopy of an apparent deep stroma corneal dystrophy: a case report

Muscular dystrophies (MDs) comprise a group of degenerative muscle disorders characterized by progressive muscle wasting and often premature death. The primary defect common to most MDs involves disruption...Full Text Available

2011-03-01

Cogan's microcystic dystrophy of the cornea: ultrastructure and photomicroscopy.

A 41-year-old white woman was referred to our Department to rule out the presence of a Fuch's corneal dystrophy. On slit-lamp biomicroscopy, small bilateral punctuate opacities appearing mostly in the...Full Text Available

Anticipation in familial lattice corneal dystrophy type I with R124C mutation in the TGFBI (BIGH3) gene

Corneal biopsy specimens from 3 patients with Cogan's microcystic corneal dystrophy were examined by light and electron microscopy. Specimens were taken from corneas showing microcysts, geographic or...Full Text Available

1978-12-01

Use of antivascular endothelial growth factor for diabetic macular edema

PurposeTo report the clinical, ophthalmic, and genetic characteristics for lattice corneal dystrophy type I (LCDI) in a Chilean family.MethodsSix...Full Text Available

Prevalence of Early and Late Age-Related Macular Degeneration in India: The INDEYE Study

BackgroundDiabetic macular edema (DME) is one of the manifestations of diabetic retinopathy leading to loss of central vision and visual acuity. It manifests itself with swelling...Full Text Available

2010-01-01

Macular abnormalities in the reattached retina.

Purpose.To estimate the prevalence of early and late age-related macular degeneration (AMD) in India.Methods.Of 7518 people aged 60 years and older...Full Text Available

2010-02-01

A comparative study between intravitreal triamcinolone and bevacizumab for macular edema due to central retinal vein occlusion with poor vision

Sixty-six patients in whom the macula was detached before surgery were observed for at least 1 year after retinal reattachment. Macular abnormalities were recognised clinically in most patients with...Full Text Available

1978-09-01

Consistency of ocular coherence tomography fast macular thickness mapping in diabetic diffuse macular edema

Aim:To compare the effect of intravitreal bevacizumab and triamcinolone in patients with macular edema after central retinal vein occlusion (CRVO), presenting with poor...Full Text Available

2011-03-01

Misfolded Proteins and Retinal Dystrophies

Objectives: To investigate optical coherence tomography consistency on foveal thickness, foveal volume, and macular volume measurements in patients with and without diffuse diabetic macular edema. Introduction: Optical coherence tomography represents an objective technique that provides cross-sectional tomographs of retinal structure in vivo. However, it is expected that poor fixation ability, as seen in diabetic macular edema, could alter its results. Several authors have discussed the reproducibility of optical coherence tomography, but only a few have addressed the topic with respect to diabetic maculopathy. Methods: The study recruited diabetic patients without clinically evident retinopathy (control group) and with diffuse macular edema (case group). Only one eye of each patient was evaluated. Five consecutive fast macular scans were taken using Ocular Coherence Tomography 3; ...

2007-07-01

Foveo-macular retinitis, solar retinopathy, and trauma.

Many mutations associated with retinal degeneration lead to the production of misfolded proteins by cells of the retina. Emerging evidence suggests that these abnormal proteins cause cell death...Full Text Available

2010-01-01

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Aphakic macular oedema following prosthetic lens implantation.

Three patients are described with foveal lesions resembling minute holes following trauma. The similarity of the lesions to foveomacular retinitis and solar retinopathy suggests that all these conditions...Full Text Available

1978-08-01

LOC387715/HTRA1 gene polymorphisms and susceptibility to age-related macular degeneration: A HuGE review and meta-analysis

Fluorescein angiography of the iris was performed on patients with plastic lens implants with cystoid oedema of the macula, and the nature of the vascular changes was compared with controls provided...Full Text Available

1977-05-01

The Dystrophin Complex Controls BK Channel Localization and Muscle Activity in Caenorhabditis elegans

PurposeTo examine the association of age-related macular degeneration (AMD) with HtrA serine peptidase 1 (HTRA1) gene rs11200638 G→A polymorphism and LOC387715/...Full Text Available

Profound human/mouse differences in alpha-dystrobrevin isoforms: a novel syntrophin-binding site and promoter missing in mouse and rat

Genetic defects in the dystrophin-associated protein complex (DAPC) are responsible for a variety of pathological conditions including muscular dystrophy, cardiomyopathy, and vasospasm. Conserved DAPC...Full Text Available

2009-12-01

Mutation Spectrum of EYS in Spanish Patients with Autosomal Recessive Retinitis Pigmentosa

BackgroundThe dystrophin glycoprotein complex is disrupted in Duchenne muscular dystrophy and many other neuromuscular diseases. The principal heterodimeric partner of dystrophin...Full Text Available

MR findings in acute Lyme disease affecting the knee. A case report

Retinitis pigmentosa (RP) is a heterogeneous group of inherited retinal dystrophies characterised ultimately by the loss of photoreceptor cells. We have recently identified a new gene (EYS) encoding...Full Text Available

2010-11-01

Visible light and risk of age-related macular degeneration.

In this paper we report a case with primarily unspecific arthralgia after surgical therapy of hallux valgus deformity and consecutive reflex sympathetic dystrophy in which MR led to the diagnosis of Lyme disease. (orig.)

2002-07-01

Mitochondrial function and redox control in the aging eye: Role of MsrA and other repair systems in cataract and macular degenerations

Sunlight exposure has been suggested as a cause of AMD. To examine this, we collected detailed histories of ocular sun exposure in 838 watermen who work on the Chesapeake Bay. The presence and severity...Full Text Available

1990-01-01

Macular and retinal nerve fiber layer thickness in Japanese measured by Stratus optical coherence tomography

Oxidative stress occurs when the level of prooxidants exceeds the level of antioxidants in cells resulting in oxidation of cellular components and consequent loss of cellular function. Oxidative...Full Text Available

2009-02-01

Inhibition of the visual cycle by A2E through direct interaction with RPE65 and implications in Stargardt disease

The purpose of this study was to determine the thickness of the macula and the retinal nerve fiber layer (RNFL) in Japanese subjects by Stratus optical coherence tomography (OCT), and to compare the...Full Text Available

2007-06-01

Experimental Approaches to the Study of A2E, a Bisretinoid Lipofuscin Chromophore of Retinal Pigment Epithelium

Stargardt disease (STGD) is the major form of inherited juvenile macular degeneration. Pyridinium bis-retinoid A2E is a major component of lipofuscin which accumulates in retinal pigment epithelium...Full Text Available

2010-10-12

Effects of Age on Optical Coherence Tomography Measurements of Healthy Retinal Nerve Fiber Layer, Macula and Optic Nerve Head

Bisretinoid lipofuscin compounds that accumulate in retinal pigment epithelial (RPE) cells are implicated in the pathogenesis of some forms of macular degeneration. In the development of approaches...Full Text Available

2010-01-01

Clinical and tomographic aspects of macular microholes; Aspectos clinicos e tomograficos dos microburacos maculares

PurposeTo determine the effects of age on global and sectoral peripapillary retinal nerve fiber layer (RNFL), macular thicknesses and optic nerve head (ONH) parameters...Full Text Available

2009-06-01

CLINICAL VALUE, NORMATIVE RETINAL SENSITIVITY VALUES, AND INTRASESSION REPEATABILITY USING A COMBINED SPECTRAL DOMAIN OPTICAL COHERENCE TOMOGRAPHY/SCANNING LASER OPHTHALMOSCOPE MICROPERIMETER

Purpose: To describe the clinical aspects and evaluate optical coherence tomography of macular microholes. Methods: Seven patients were assessed (8 eyes) with microholes of the macula. All patients underwent complete eye examination, fundus photography, fluorescent angiography and OCT-3 imaging. Results: Ages ranged from 26 to 69 years. Six patients were female (85.7%) and five of them had microhole in the right eye. The presenting symptom was decrease in visual acuity (71.3%) and central scotoma in (14.3%). Five eyes (71.4%) had no defects shown by fluorescent angiography. A defect in the outer retina was demonstrated in all eyes on optical coherence tomography. The lesions were nonprogressive. Conclusion: Macular microholes are small lamellar defects in the outer retina. The condition is nonprogressive, generally unilateral and compatible with good visual acuity. Fundus biomicroscopy associated with an optical coherence tomography are the ...

2009-07-01

Pseudophakic and aphakic retinal detachment mimicking cystoid macular edema.

PurposeTo establish normative values for macular light sensitivity and to determine the intrasession fluctuation of perimetric responses using the OPKO/OTI microperimeter.Full Text Available

2011-02-01

Different phenotypes of lattice corneal dystrophy type I in patients with 417C>T (R124C) and 1762A>G (H572R) mutations in TGFBI (BIGH3)

Seventeen eyes of patients who were being followed by their ophthalmologists for aphakic (8 eyes) or pseudophakic (9 eyes) cystoid macular edema (CME) were examined on referral. All eyes showed retinal detachment (RD), and eight eyes had CME with RD. Most RDs were inferior and shallow. Proliferative vitreoretinopathy (PVR) was noted in 15 eyes (88.2%). There was history of vitreous loss during the last intraocular surgery in 88.2% eyes. Visual acuity ranged from 20/40 to hand movements. Fundus view was hazy in most eyes, and indirect ophthalmoscopy was most useful in detecting RD. Retinal reattachment with scleral buckling and/or vitrectomy was achieved in 16 eyes (94.1%). Postoperative visual recovery was jeopardized due to longstanding macular detachment of greater than 1 month in 11 eyes (64.7%). Eight eyes (47%) achieved 20/50 vision whereas 25% did not improve to better than 20/400. Factors leading to failure in making the correct ...

1987-07-01

Comparison of 25- and 23-gauge sutureless microincision vitrectomy surgery in the treatment of various vitreoretinal diseases

PurposeTo describe clinical data and to characterize mutations in the transforming growth factor beta-induced (TGFBI) gene in patients from three unrelated Chilean...Full Text Available

British Library Electronic Table of Contents (United Kingdom)

PurposeTo compare the effectiveness and safety of 25- and 23-gauge sutureless microincision vitrectomy surgery (MIVS) in the management of various vitreoretinal diseases.MethodsEighty-five consecutive patients undergoing sutureless vitrectomy during January to April 2008 were randomized to either 25- or 23-gauge MIVS. Data collected prospectively included best-corrected visual acuity (BCVA), operation time, postoperative visual recovery, postoperative anterior segment change, and complications.ResultsThe most common indications for MIVS were macular hole, macular pucker, vitreous haemorrhage, and diabetic traction retinal detachment. Mean operation times of the 25-gauge (n=38) and 23-gauge groups (n=47) were 33.68 and 34.47?min, respectively (P=0.942). Mean BCVA improved significantl...

2010-01-01

Visual recovery after vitrectomy for macular hole using 25-gauge instruments

British Library Electronic Table of Contents (United Kingdom)

Abstract. Purpose: To determine whether vitrectomy with 25-gauge instruments contributes to better postoperative visual recovery after macular hole (MH) surgery. Methods: The medical records for 46 consecutive eyes operated for MH by a single surgeon were retrospectively examined. Vitrectomy had been performed with a 25-gauge instrument in 23 eyes (25-G group) and with a 20-gauge instrument in 23 eyes (20-G group). Postoperative visual acuity (VA) in logMAR (logarithm of the minimum angle of resolution) units after 1 week and 1, 3, 6, 9 and 12 months, operating time, and volume of intraocular irrigating fluid were compared between the two groups. Results: Mean preoperative logMAR VA was 0.72 in the 25-G group and 0.68 in the 20-G group (p = 0.282, unpaired t-test). One week after surgery, ...

2008-01-01

Hypogonadism in DM1 and its relationship to erectile dysfunction

British Library Electronic Table of Contents (United Kingdom)

Myotonic dystrophy type 1 (DM1) is characterized by both a premature appearance of age-related phenotypes and multiple organ involvement, which affects skeletal and smooth muscle as well as the eye, heart, central nervous system, and endocrine system. Although erectile dysfunction (ED) is a frequent complaint in patients with DM1, it has not been investigated in great depth. Hypogonadism, which is reported to be one of the physical causes of ED in the general population, frequently occurs in DM1. We planned this case?control study to evaluate the relationship between hypogonadism, as defined by the sexual hormone profile (FSH, LH, testosterone (T) and prolactin) and ED, as assessed by means of an internationally validated self-administered questionnaire (IIEF). DM1 patients had significant...

2011-01-01

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Vitrectomy Timing for Retained Lens Fragments After Surgery for Age-Related Cataracts: A Systematic Review and Meta-Analysis

British Library Electronic Table of Contents (United Kingdom)

PurposeTo evaluate the effect of vitrectomy timing on outcomes for patients with crystalline retained lens fragments receiving vitrectomy 3+ days after cataract surgery. DesignSystematic review and meta-analysis of retrospective interventional cases series. MethodsSearches of MEDLINE (English, 1/1/85 through 7/30/2010) and article reference lists. Articles were screened for patients with crystalline retained lens fragments after surgery for age-related cataracts, discussion of vitrectomy timing, and, for the meta-analysis, patient totals for at least 1 outcome and multiple time periods, 10+ patients, and mean follow-up ?3 months. Outcomes included visual acuity, retinal detachment, increased intraocular pressure, intraocular infection/inflammation, cystoid macular edema, and corneal...

2011-01-01

Ultraviolet-B Phototoxicity and Hypothetical Photomelanomagenesis: Intraocular and Crystalline Lens Photoprotection

British Library Electronic Table of Contents (United Kingdom)

Purpose Ultraviolet-B (UV-B) radiation can cause phototoxic macular injuries in young people who have been sunbathing but not sungazing and in welders. Welders have a reportedly increased risk of uveal melanoma. We analyze phakic and pseudophakic risks for solar and welding arc UV-B exposure. Design Optical radiation measurement, analysis, and perspective. Methods Spectral transmittances were measured for UV-transmitting, UV-blocking, and blue-blocking intraocular lenses (IOLs). The photoprotective performances of crystalline and intraocular lenses were analyzed using relevant epidemiologic and laboratory data and action spectra for acute retinal phototoxicity and melanoma photocarcinogenesis. Results Crystalline lens UV-B retinal protection is deficient in children and young adults, incre...

2010-01-01

Post harvest improvement of zeaxanthin content of vegetables

British Library Electronic Table of Contents (United Kingdom)

Zeaxanthin is a carotenoid produced by plants and has been associated with protection of the photosynthetic machinery under light stress and, together with lutein, in protection of the central retina of the eye. Zeaxanthin levels in blood plasma have been negatively correlated to the development of AMD (age-related macular degeneration) (Gale et al., 2003). Under normal conditions, plants have a low content of zeaxanthin. The aim of this study was to increase the zeaxanthin content in green vegetables by post harvest treatments. Efficient conditions for activation of the endogenous enzyme system generating zeaxanthin was established and included incubation at low pH (2.5-5.5), with the membrane permeable acetic acid/acetate buffer at room temperature or above for 30min or more. Typically m...

2010-01-01

The treatment of chronic pain by epidural spinal cord stimulation--a 15 year follow up; present status.

Soil Redox Chemistry Limitation of Selenium Concentration in Carex Species Sedges

Pain is necessary for survival but chronic pain is disabling and causes significant health and economic problems. This study provides an understanding of the future for spinal cord stimulation. Stimulation by means of chronically implanted electrodes, was carried out in 200 patients with pain of varied benign organic etiology. In 177 of them, pain was confined to the failed back syndrome. Most patients were referred by a Pain Management Service. 226 epidural implants were used: 80 unipolar, 59 Resume, 12 bipolar, and 75 quadripolar. Patients were followed for periods of 6 months to 12 years, with a mean follow-up of 44 months. 84 patients (42%) were able to control their pain by stimulation alone, 22 patients (11%) needed occasional analgesic supplements along with their stimulation program. Pain secondary to failed back syndrome, multiple sclerosis, peripheral vascular disease, sympathetic dystrophy and diabetic neuropathy responded favorably. Pain due to cauda ...

1997-06-01

SE and I content in military total diet

The trace element selenium (Se) is required in the production of enzymes that protect mammalian cells from oxidative damage due to the byproducts of aerobic respiration. Its deficiency in livestock results in the nutritional muscular dystrophy called white muscle disease. This especially affects juveniles in the preweaning period. Symptoms have also been reported in wild herbivores on low-Se forage, and their appearance may be episodic, suggesting temporal variations in Se uptake by plants. Here, we report variations in selenium concentrations in Carex spp. sedges used as forage by bighorn sheep (Ovis canadensis) on summer range in the Wyoming, Wind River Mountains, and correlate those variations with soil conditions that affect the bioavailability of selenium. Variations in available Se are explained based upon the known oxidation/reduction chemistry of the element. It is concluded that water-saturated, alpine soils provide conditions suitable for the reduction of ...

2007-09-01

International Nuclear Information System (INIS)

Selenium and iodine are trace elements that play an important role in health of man and animals. Concerning supply of both elements it is important to meet nutritional needs. According to discoveries selenium is a part of an active group of glutathione peroxidase enzyme. It prevents necrotic changes in liver, muscle dystrophy and has impact on growth. Extensive studies on content of selenium in plant and animal origin products were started in the beginning of the seventies in Western Europe (Frankenberger, et al., 1998). The aim of our work was to determine selenium and iodine content in total diets of Slovene military. Slovene military nutrition is based on its own nutritional standards, which determine 200 #mu#g of iodine and 50 #mu#g of selenium in daily diet (Rep. Slovenija Ministrstvo za Obrambo, 1994). To determine if the amounts of both elements meet the standards we have analysed 20 total daily diets from four different barracks within a two month period. ...

2003-06-10

Stability of lutein in wholegrain bakery products naturally high in lutein or fortified with free lutein.

Celastrol regulates innate immunity response via NF-@kB and Hsp70 in human retinal pigment epithelial cells

Lutein is a yellow pigment found in common foods that promotes the health of eyes and skin and is associated with reduced risk of age-related macular degeneration and cataracts. In the present study, selected high-lutein wheat and corn were milled into wholegrain flours by two mills to improve flour uniformity. The high-lutein and lutein-fortified wholegrain flours were processed into breads, cookies, and muffins to study lutein stability during baking and subsequent storage. Lutein and its isomers were separated, identified, and quantified by LC-UV/vis and LC-MS following extraction with water-saturated 1-butanol. Baking resulted in a significant reduction in all-trans-lutein and the formation of cis-lutein and cis-zeaxanthin isomers. Subsequent storage at ambient temperature had a slight impact on the content of all-trans-lutein. Effects of processing were more pronounced in lutein-fortified products, and the degradation rate of lutein was influenced by ...

2010-09-22

British Library Electronic Table of Contents (United Kingdom)

Elevated nuclear factor kappa B (NF-@kB) activity and interleukin-6 (IL-6) secretion participates in the pathology of several age and inflammatory-related diseases, including age-related macular degeneration (AMD), in which retinal pigment epithelial cells are the key target. Recent findings reveal that heat shock protein 70 (Hsp70) may affect regulation of NF-@kB. In the current study, effects of Hsp70 expression on NF-@kB RelA/p65 activity were evaluated in human retinal pigment epithelial cells (ARPE-19) by using celastrol, a novel anti-inflammatory compound. Anti-inflammatory properties of celastrol were determined by measuring expression levels of IL-6 and endogenous NF-@kB levels during lipopolysaccharide (LPS) exposure by using enzyme-linked immunosorbent assays (ELISA). Cell viabil...

2011-01-01

Evolution of ASTEC V1.2 rev.1 code for WWER-1000 reactors/SBO sequence

International Nuclear Information System (INIS)

In this paper a comparison between calculations of severe accidents occurred from WWER-1000 with ASTEC code specified for an event of full unloading with relief valves stuck opened with no hydroaccumulators intervention is presented. The purpose of the analyses provided is to present the relationship between the improvements of the actual version (ASTEC Vl.2 rev. 1) and ASTEC V1.1 p2 like: code modifications, incoming data improvements. Such discrepancies are to be examined. Case by case suggestions for ASTEC improvements are to be provided.

2006-06-14

Cloning of human basic A1, a distinct 59-kDa dystrophin-associated protein encoded on chromosome 8q23-24

Dose distribution in water for monoenergetic photon point sources in the energy range of interest in brachytherapy: Monte Carlo simulations with PENELOPE and GEANT4

Duchenne and Becker muscular dystrophies are caused by defects of dystrophin, which forms a part of the membrane cytoskeleton of specialized cells such as muscle. It has been previously shown that the dystrophin-associated protein A1 (59-kDa DAP) is actually a heterogeneous group of phosphorylated proteins consisting of an acidic ({alpha}-A1) and a distinct basic ({beta}-A1) component. Partial peptide sequence of the A1 complex purified from rabbit muscle permitted the design of oligonucleotide probes that were used to isolate a cDNA for one human isoform of A1. This cDNA encodes a basic A1 isoform that is distinct from the recently described syntrophins in Torpedo and mouse and is expressed in many tissues with at least five distinct mRNA species of 5.9, 4.8, 4.3, 3.1, and 1.5 kb. A comparison of the human cDNA sequence with the GenBank expressed sequence tag (EST) data base has identified a relative from human skeletal muscle, EST25263, which is probably a human ...

1994-05-10

CERN Document Server

Monte Carlo calculations using the codes PENELOPE and GEANT4 have been performed to characterize the dosimetric properties of monoenergetic photon point sources in water. The dose rate in water has been calculated for energies of interest in brachytherapy, ranging between 10 keV and 2 MeV. A comparison of the results obtained using the two codes with the available data calculated with other Monte Carlo codes is carried out. A chi2-like statistical test is proposed for these comparisons. PENELOPE and GEANT4 show a reasonable agreement for all energies analyzed and distances to the source larger than 1 cm. Significant differences are found at distances from the source up to 1 cm. A similar situation occurs between PENELOPE and EGS4.

2006-01-01

cDNA cloning, sequence analysis and organ distribution of horse preproendothelin-2.

Synthesis of pellet-type red mud adsorbents for removal of heavy metal ions

We cloned and characterized horse preproendothelin-2 (PPET-2) cDNA from intestinal tissue. The cDNA encoded 178 amino acids of the PPET-2 polypeptide, in which a 21-amino-acid mature endothelin-2 peptide and a 16-amino acid endothelin-2-like peptide were found. For the open reading frame the correspondence of horse PPET-2 cDNA with those of the ferret, human, dog, mouse and rat was 85.1%, 84.9%, 82.1%, 77.8% and 77.2%, respectively. Analysis of the organ distribution of PPET-2 mRNA by reverse transcription-polymerase chain reaction demonstrated that the kidney, stomach and small intestine are major sites of expression of the PPET-2 gene. Surprisingly, the mRNA is not detected in the large intestine, where high expression is demonstrated in the mouse and rat. This difference may result from the underlying functional differences of the large intestine between a herbivore (horse) and an omnivore (mouse and rat). PMID:15838341

2004-11-01

Structural Insights into the Interaction of the Evolutionarily Conserved ZPR1 Domain Tandem with Eukaryotic EF1A, Receptors, and SMN Complexes

Red mud is generated as a by-product in the production of Al(OH){sub 3}/Al{sub 2}O{sub 3} from bauxite ore. In this study the pellet-type adsorbents have been made from the red mud, and their adsorption capacities of heavy metal ions have been tested. The pellet-type adsorbents were synthesized to utilize the excellent adsorption capacity of the powder-type adsorbent for industrial application. The pellet-type adsorbents were prepared by mixing several kinds of additives with the red mud. It is found that the pellet-type adsorbent, made by sintering a mixture of red mud (96.0 wt%), polypropylene (2.5 wt%), fly ash (0.5 wt%), and sodium meta-silicate (1.0 wt%) at 1200 deg.C for 30 minutes, has the highest adsorption capacity. In this work, the two kinds of pellet-type adsorbents (bead-type, crushed-type) were prepared. The crushed-type adsorbent was found to show a better adsorption/desorption performance than the bead-type adsorbent. The crushed-type adsorbent showed a good adsorption ...

2000-02-28