Residual laminin-binding activity and enhanced dystroglycan glycosylation by LARGE in novel model mice to dystroglycanopathy

Hypoglycosylation and reduced laminin-binding activity of α-dystroglycan are common characteristics of dystroglycanopathy, which is a group of congenital and limb-girdle muscular dystrophies. Fukuyama-type congenital muscular dystrophy (FCMD), caused by a mutation in the fukutin gene, is a severe fo...

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Autors principals: Kanagawa, Motoi, Nishimoto, Akemi, Chiyonobu, Tomohiro, Takeda, Satoshi, Miyagoe-Suzuki, Yuko, Wang, Fan, Fujikake, Nobuhiro, Taniguchi, Mariko, Lu, Zhongpeng, Tachikawa, Masaji, Nagai, Yoshitaka, Tashiro, Fumi, Miyazaki, Jun-Ichi, Tajima, Youichi, Takeda, Shin'ichi, Endo, Tamao, Kobayashi, Kazuhiro, Campbell, Kevin P., Toda, Tatsushi
Format: Artigo
Idioma:Inglês
Publicat: Oxford University Press 2009
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Articles
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC2638827/
https://ncbi.nlm.nih.gov/pubmed/19017726
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1093/hmg/ddn387
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