Publication:
Reduced expression of sarcospan in muscles of Fukuyama congenital muscular dystrophy

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dc.contributor.authorYoshihiro Wakayamaes
dc.contributor.authorMasahiko Inouees
dc.contributor.authorHiroko Kojima
dc.contributor.authorSumimasa Yamashita
dc.contributor.authorSeiji Shibuya
dc.contributor.authorTakahiro Jimi
dc.contributor.authorHajime Hara
dc.contributor.authorYoko Matsuzaki
dc.contributor.authorHiroaki Oniki
dc.contributor.authorMotoi Kanagawa
dc.contributor.authorKazuhiro Kobayashi
dc.contributor.authorTatsushi Toda
dc.date.accessioned2013-01-18T13:44:53Z
dc.date.available2013-01-18T13:44:53Z
dc.date.issued2008
dc.description.abstractSummary. Expression profiles of sarcospan in muscles with muscular dystrophies are scarcely reported. To examine this, we studied five Fukuyama congenital muscular dystrophy (FCMD) muscles, five Duchenne muscular dystrophy (DMD) muscles, five disease control and five normal control muscles. Immunoblot showed reactions of sarcospan markedly decreased in FCMD and DMD muscle extracts. Immunohistochemistry of FCMD muscles showed that most large diameter myofibers expressed sarcospan discontinuously at their surface membranes. Immature small diameter FCMD myofibers usually did not express sarcospan. Immunoreactivity of sarcospan in DMD muscles was similarly reduced. With regard to dystroglycans and sarcoglycans, immunohistochemistry of FCMD muscles showed selective deficiency of glycosylated α- dystroglycan, together with reduced expression of ß- dystroglycan and α-, ß-, γ-, δ-sarcoglycans. Although the expression of glycosylated α-dystroglycan was lost, scattered FCMD myofibers showed positive immunoreaction with an antibody against the core protein of α-dystroglycan. The group mean ratios of sarcospan mRNA copy number versus GAPDH mRNA copy number by real-time RT-PCR showed that the ratios between FCMD and normal control groups were not significantly different (P>0.1 by the two-tailed t test). This study implied either O-linked glycosylation defects of α-dystroglycan in the Golgi apparatus of FCMD muscles may lead to decreased expression of sarcoglycan and sarcospan molecules, or selective deficiency of glycosylated α-dystroglycan due to impaired glycosylation in FCMD muscles may affect the molecular integrity of the basal lamina of myofibers. This, in turn, leads to decreased expression of sarcoglycans, and finally of sarcospan at the FCMD myofiber surfaces.es
dc.formatapplication/pdfes
dc.format.extent14es
dc.identifier.issn0213-3911es
dc.identifier.urihttp://hdl.handle.net/10201/29729
dc.languageenges
dc.publisherMurcia : F. Hernándezes
dc.relation.ispartofHistology and histopathologyes
dc.rightsinfo:eu-repo/semantics/openAccesses
dc.subjectSarcospanes
dc.subjectMuscular dystrophyes
dc.subject.other615 - Farmacología. Terapéutica. Toxicología. Radiologíaes
dc.titleReduced expression of sarcospan in muscles of Fukuyama congenital muscular dystrophyes
dc.typeinfo:eu-repo/semantics/articlees
dspace.entity.typePublicationes
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Histology and histopathology Vol.23,nº12 (2008)