Restoration of Functional Glycosylation of α-Dystroglycan in FKRP Mutant Mice Is Associated with Muscle Regeneration.

@article{Awano2015RestorationOF,
  title={Restoration of Functional Glycosylation of α-Dystroglycan in FKRP Mutant Mice Is Associated with Muscle Regeneration.},
  author={Hiroyuki Awano and Anthony Blaeser and Elizabeth Keramaris and Lei Xu and Jason Tucker and Bo Wu and Pei Juan Lu and Qi Long Lu},
  journal={The American journal of pathology},
  year={2015},
  volume={185 7},
  pages={2025-37}
}
Mutations in fukutin-related protein (FKRP) gene are characterized with lack of functionally glycosylated α-dystroglycan (F-α-DG). Surprisingly, a few muscle fibers express strong F-α-DG. Herein, we investigated the restoration of F-α-DG in the FKRP mutant muscles and showed that the restoration of glycosylation is associated with muscle regeneration and dependent on the expression of both like-glycosyltransferase (LARGE) and partially functional FKRP. F-α-DG in the regenerating fibers reaches… CONTINUE READING