Efficient engraftment of pluripotent stem cell-derived myogenic progenitors in a novel immunodeficient mouse model of limb girdle muscular dystrophy 2I

Efficient engraftment of pluripotent stem cell-derived myogenic progenitors in a novel immunodeficient mouse model of limb girdle muscular dystrophy 2I

Abstract Background Defects in α-dystroglycan (DG) glycosylation characterize a group of muscular dystrophies known as dystroglycanopathies. One of the key effectors in the α-DG glycosylation pathway is the glycosyltransferase fukutin-related protein (FKRP). Mutations in FKRP lead to a large spectru...

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Bibliographic Details
Main Authors: Karim Azzag, Carolina Ortiz-Cordero, Nelio A. J. Oliveira, Alessandro Magli, Sridhar Selvaraj, Sudheer Tungtur, Weston Upchurch, Paul A. Iaizzo, Qi Long Lu, Rita C. R. Perlingeiro
Format: Article
Language:English
Published: BMC 2020-04-01
Series:Skeletal Muscle
Subjects:
Muscular dystrophy
FKRP
LGMD2I
Pluripotent stem cells
Transplantation
Muscle regeneration
Online Access:http://link.springer.com/article/10.1186/s13395-020-00228-3

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http://link.springer.com/article/10.1186/s13395-020-00228-3

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