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Thursday, July 30, 2020 | History

2 edition of Dystrophin expression in the retina. found in the catalog.

Dystrophin expression in the retina.

Vinita N. D"Souza

Dystrophin expression in the retina.

by Vinita N. D"Souza

  • 103 Want to read
  • 32 Currently reading

Published by National Library of Canada in Ottawa .
Written in English


Edition Notes

Thesis (M.Sc.) -- University of Toronto, 1995.

SeriesCanadian theses = -- Thèses canadiennes
The Physical Object
Pagination2 microfiches : negative. --
ID Numbers
Open LibraryOL17889379M
ISBN 100612075842

Absent dystrophin muscle (M) isoform expression; Increased expression of brain (B) & cerebellar Purkinje (CP) isoforms. Upregulated by dystrophin muscle enhancer 1 (DME1) 4 Muscle-specific enhancer in dystrophin gene; B & CP isoforms are full length ( kDa) like the M isoform Intron Alu-like sequence rearrangement Alu-like sequence: bp. Europe PMC is an archive of life sciences journal literature. Expression patterns of dystrophin products, especially of apodystrophin-1/Dp71, in the neural retina of Amphib ian urodele Pleurodeles elizrosshubbell.com: Arsanto JP.

Oct 05,  · Restoration of dystrophin expression was accompanied by improved muscle histology. Science, this issue p. [86][1] Mutations in the gene encoding dystrophin, a protein that maintains muscle integrity and function, cause Duchenne muscular dystrophy (DMD).Cited by: Expression. Dystroglycan is widely distributed in non-muscle tissues as well as in muscle tissues. During epithelial morphogenesis of kidney, the dystroglycan complex is shown to act as a receptor for the basement membrane. Dystroglycan expression in Mus musculus brain and neural retina Aliases: DAG1, DAG, A3a, AGRNR, DAG, MDDGC7, .

Our current understanding suggests that it is the unknown glial role of brain dystrophin, rather than its GABAergic synaptic role, which is critical for cognitive function. We are, however, limited by lack of clarity regarding both the expression of some of the intermediate isoforms in the brain and the true core function of the dystrophin. We have previously shown that the deletion of the dystrophin Dp71 gene induces a highly permeable blood retinal barrier (BRB). Given that BRB breakdown is involved in retinal inflammation and the.


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Dystrophin expression in the retina by Vinita N. D"Souza Download PDF EPUB FB2

May 01,  · Dystrophin expression in the human retina is required for normal function as defined by electroretinography Skip to main content Thank you for visiting elizrosshubbell.com Cited by: dystrobrevin expression in the retina yet, but recently our data demonstrate that dystrobrevin is expressed at the paravitreous and perivascular regions, in the ILM, OPL and retinal pigment epithelium (RPE) of the retina (manuscript in submission).

Dystrophin and DAPs expression in the retina is summarized in Table 1. Since its description, the dystrophin-glycoprotein complex has emerged as an important structural unit of muscle and also as a critical nexus for understanding muscular dystrophies arising from defects in several distinct elizrosshubbell.com by: 5.

Temporal and spatial expression patterns of dystrophin gene products in the retina. (A) Western blot analysis of dystrophin gene products shows that the retina expresses at least 4 different dystrophin gene products at similar levels (Dp, Dp, Dp and Dp71), while brain and muscle tissue predominantly express 1 gene elizrosshubbell.com: Felicitas Bucher, Felicitas Bucher, Mollie S.H.

Friedlander, Edith Aguilar, Toshihide Kurihara, Tim. analyzing the expression patterns of dystrophin isoforms in the developing and adult newt retina using Dpspecific 5F3 mAb in addition to H-5A3 mAb.

Western blot analysis showed the pre-sence in adult newt retina of dystrophin family isoforms, i.e., Cited by: 2. In this report, we compare the temporal and regional expression patterns of dystrophin and Dp71 at different stages of embryonic development and during retinal differentiation in zebrafish.

The Dp71 transcripts are the earliest to be expressed at 9–10 h post-fertilization (hpf) in the axial elizrosshubbell.com by: Dystrophin is primarily expressed in skeletal, cardiac, and smooth muscle cells, with Dystrophin expression in the retina.

book amounts expressed in the brain and retina. Isoforms of dystrophin, which are smaller in size, are expressed in nearly all tissues examined.

Dystrophin Dp71 is expressed in all tissues, with the exception of skeletal muscle, and is the main Duchenne muscular dystrophy (DMD) gene product in brain. Oct 13,  · Last week, I criticized Sarepta Therapeutics for failing to disclose critical dystrophin expression data regarding their exon-skipping drug candidate eteplirsen for the treatment of DMD.

Instead, they merely disclosed the percentage of muscle fibers staining positive for dystrophin, i.e. fibers that expressed some dystrophin, which, of course, would be a much more impressively sounding Author: Dirk Haussecker. The dystrophin gene is the largest gene described in human beings, it spans more than 2·5 million bp of genomic sequence, which correspond to about 0·1% of the total human genome1, 2, 3 or about 1·5% of the entire × chromosome.

99% of the gene for dystrophin is made of introns; and the coding sequence is 86 exons (including seven promoters linked to unique first exons).Cited by: Amplitudes and implicit times were measured for a-waves, b-waves, and digitally filtered oscillatory elizrosshubbell.commdx Cv5 mice, with mutations near the amino terminus and lacking expression of Dp, had ERGs similar to those of C57BL/6J elizrosshubbell.com Cv2 andmdx Cv4 mice, with mutations in the center of dystrophin and who do not express isoforms Dp, Dp, or Dp (mdx Cv4), had Cited by: The expression patterns of the DMD (Duchenne Muscular Dystrophy) gene products, especially of Dp71 (apodystrophin-1) were investigated by immunofluorescence and immunoblotting in the retina of the Amphibian urodele Pleurodeles waltl.

H-5A3 monoclonal antibody (mAb), directed against the C-terminal region of dystrophin/utrophin, and 5F3 mAb, directed against the last 31 amino acids of Cited by: 2. rna and protein expression summary i Below is an overview of RNA and protein expression data generated in the Human Protein Atlas project.

Analyzed tissues are divided into color-coded groups according to which functional features they have in common. Dystrophin is a protein located between the sarcolemma and the outermost layer of myofilaments in the muscle fiber.

It is a cohesive protein, linking actin filaments to other support proteins that reside on the inside surface of each muscle fiber's plasma membrane (sarcolemma).Aliases: DMD, BMD, CMD3B, DXS. Jun 07,  · Duchenne muscular dystrophy (DMD; OMIM ) is an X-linked recessive disorder that affects 1 in 3, males and is caused by mutations in the dystrophin gene (Blake et al, ).

The gene is the largest in the human genome, encompassing million Cited by: Dystroglycan Expression in the Retina The absence of dystrophin in DMD and in one of its animal models, the rndx mouse, leads to a reduction of expression of some members of the DGC.

Sep 01,  · At P5, the outer plexiform layer has begun to form in the central retina, as judged by dystrophin (Dys) expression (A), At P10, the entire retina has developed an outer plexiform layer in which dystrophins are prominently expressed (Fig.

10 A).Cited by: Jun 10,  · Introduction. Dp71 belongs to the dystrophin superfamily, and is a short product of the DMD gene, located on the X chromosome, involved in Duchenne muscular dystrophy (DMD) ().Dp71—a membrane-bound cytoskeletal protein ()—plays an important role in glial and vascular functions in the elizrosshubbell.com forms, with other proteins called dystrophin associated proteins (DAPs), a dystrophin Cited by: 9.

The abnormal retinal neurotransmission observed in Duchenne muscular dystrophy (DMD) patients and in some genotypes of mice lacking dystrophin has been attributed to altered expression of short.

Duchenne muscular dystrophy (DMD), a rare genetic disorder characterized by progressive muscle weakness, is caused by the absence or a decreased amount of the muscle cytoskeletal protein dystrophin. Currently, several therapeutic approaches to cure DMD are being investigated, which can be categorized into two groups: therapies that aim to restore dystrophin expression, and those that aim Cited by:.

Dystrophin and Its Isoforms. and that a severe reduction of dystrophin has no gross effect on retinal structure, but could influence intraretinal signalling at the level of the photoreceptor.Apr 13,  · In the retina, dystrophin is expressed at the level of the outer plexiform layer (Dp) in the inner limiting membrane (Dp71).

7 – 12 Dp is also found at the cone pedicle, in the region of the ribbon synapse. 13 Electrophysiological studies showed that Dp is essential for the physiology of the retina, since patients with DMD and deletions downstream of exon 30 had serious impairment in both Cited by: Oct 01,  · DMD, the largest known human gene, provides instructions for making a protein called elizrosshubbell.com protein is located primarily in muscles used for movement (skeletal muscles) and in heart (cardiac) muscle.

Small amounts of dystrophin are present in nerve cells in the brain.