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Current knowledge of dystrophin and dystrophin-associated proteins in the retina

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dc.contributor.authorUeda, H.es
dc.contributor.authorBaba, T.es
dc.contributor.authorOhno, S.
dc.date.accessioned2011-02-22T11:21:55Z
dc.date.available2011-02-22T11:21:55Z
dc.date.issued2000
dc.description.abstractDramatical development of molecular genetics has been disclosing the molecular mechanism of Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (RMD). DMD gene product, dystrophin, is a submembranous cytoskeletal protein and many dystrophin-associated proteins (DAPs) have been identified, such as utrophin, dystroglycans, sarcoglycans, syntrophins and dystrobrevins. Dystrophin and DAPs are very important proteins not only for skeletal, cardiac, or smooth muscles but also for peripheral and central nervous systems including the retina. The retina has been extensively examined to demonstrate that dystrophin and B-dystroglycan localize at the photoreceptor terminal, and their deficiency produces the abnormal neurotransmission between photoreceptor cells and ON-bipolar cells. Dystrophin has seven isoforms in variable tissues, and the retina contains fulllength dystrophin (Dp427), Dp260, and Dp71. Recent studies have demonstrated that Dp71 localizes in the inner limiting membrane (INL) and around the blood vessel, and Dp260 is expressed in the outer plexiform layer (OPL). B-dystroglycan is also expressed in the same regions as well as dystrophin, but it remains unclear whether other DAPs are expressed in the retina or not. It is generally assumed that dystrophin functions to stabilize muscle fibers with DAPs by linking the sarcolemma to the basement membrane, but its function in the retina is totally unknown so far.es
dc.formatapplication/pdfes
dc.format.extent8es
dc.identifier.issn0213-3911es
dc.identifier.urihttp://hdl.handle.net/10201/19336
dc.languageenges
dc.publisherMurcia : F. Hernándezes
dc.relation.ispartofHistology and histopathologyes
dc.rightsinfo:eu-repo/semantics/openAccesses
dc.subjectRetinaes
dc.subjectDystrophines
dc.subject.otherCDU::6 - Ciencias aplicadas::61 - Medicina::611 - Anatomíaes
dc.titleCurrent knowledge of dystrophin and dystrophin-associated proteins in the retinaes
dc.typeinfo:eu-repo/semantics/articlees
dspace.entity.typePublicationes
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Histology and histopathology Vol.15, nº 3 (2000)