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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...
Autores principales: | Cataldi, Marcela P., Blaeser, Anthony, Lu, Peijuan, Leroy, Victoria, Lu, Qi Long |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
American Society of Gene & Cell Therapy
2019
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Materias: | |
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 |
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