| First Author | Awano H | Year | 2015 |
| Journal | Am J Pathol | Volume | 185 |
| Issue | 7 | Pages | 2025-37 |
| PubMed ID | 25976249 | Mgi Jnum | J:223307 |
| Mgi Id | MGI:5648656 | Doi | 10.1016/j.ajpath.2015.03.017 |
| Citation | Awano H, et al. (2015) Restoration of Functional Glycosylation of alpha-Dystroglycan in FKRP Mutant Mice Is Associated with Muscle Regeneration. Am J Pathol 185(7):2025-37 |
| abstractText | Mutations in fukutin-related protein (FKRP) gene are characterized with lack of functionally glycosylated alpha-dystroglycan (F-alpha-DG). Surprisingly, a few muscle fibers express strong F-alpha-DG. Herein, we investigated the restoration of F-alpha-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-alpha-DG in the regenerating fibers reaches up to normal levels and lasts for >4 weeks, but no up-regulation of the LARGE and FKRP is detected during the regeneration process. The FKRP protein with P448L mutation is sufficient for functional glycosylation of alpha-DG in regenerating fibers, but not in mature fibers. Thus, factors other than FKRP enable regenerating fibers to produce functional alpha-DG, compensating for the defect in FKRP function. Identification of factors other than LARGE and FKRP could generate new approaches for restoration of F-alpha-DG in mature muscle fibers with defects in FKRP functions. |
