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Título: Reduced expression of sarcospan in muscles of Fukuyama congenital muscular dystrophy
Fecha de publicación: 2008
Editorial: Murcia : F. Hernández
ISSN: 0213-3911
Materias relacionadas: 615 - Farmacología. Terapéutica. Toxicología. Radiología
Palabras clave: Sarcospan
Muscular dystrophy
Resumen: Summary. 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.
Autor/es principal/es: Yoshihiro Wakayama
Masahiko Inoue
Hiroko Kojima
Sumimasa Yamashita
Seiji Shibuya
Takahiro Jimi
Hajime Hara
Yoko Matsuzaki
Hiroaki Oniki
Motoi Kanagawa
Kazuhiro Kobayashi
Tatsushi Toda
Forma parte de: Histology and histopathology
URI: http://hdl.handle.net/10201/29729
Tipo de documento: info:eu-repo/semantics/article
Número páginas / Extensión: 14
Derechos: info:eu-repo/semantics/openAccess
Aparece en las colecciones:Vol.23,nº12 (2008)

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