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ISPD Overexpression Enhances Ribitol-Induced Glycosylation of α-Dystroglycan in Dystrophic FKRP Mutant Mice

Dystroglycanopathy, a subgroup of muscular dystrophies, is characterized by hypoglycosylation of α-dystroglycan (α-DG), which reduces its laminin-binding activity to extracellular matrix proteins, causing progressive loss of muscle integrity and function. Mutations in the fukutin-related protein (FK...

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Principais autores: Marcela P. Cataldi, Anthony Blaeser, Peijuan Lu, Victoria Leroy, Qi Long Lu
Format: Artigo
Sprog:Inglês
Udgivet: Elsevier 2020-06-01
Serier:Molecular Therapy: Methods & Clinical Development
Fag:
FKRP
ISPD
ribitol
glycosylation
muscular dystrophy
Online adgang:http://www.sciencedirect.com/science/article/pii/S2329050119301548
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