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...

Descripción completa

Detalles Bibliográficos
Autores principales: Cataldi, Marcela P., Blaeser, Anthony, Lu, Peijuan, Leroy, Victoria, Lu, Qi Long
Formato: Online Artículo Texto
Lenguaje:English
Publicado: American Society of Gene & Cell Therapy 2019
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6970132/
https://www.ncbi.nlm.nih.gov/pubmed/31988979
http://dx.doi.org/10.1016/j.omtm.2019.12.005

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6970132/
https://www.ncbi.nlm.nih.gov/pubmed/31988979
http://dx.doi.org/10.1016/j.omtm.2019.12.005

Ejemplares similares