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Article

Abnormal Skeletal Muscle Regeneration plus Mild Alterations in Mature Fiber Type Specification in Fktn-Deficient Dystroglycanopathy Muscular Dystrophy Mice.

PloS one - 1 Jan 2016

Foltz Steven J, Modi Jill N, Melick Garrett A, Abousaud Marin I, Luan Junna, Fortunato Marisa J, Beedle Aaron M

Abstract excerpt

Glycosylated α-dystroglycan provides an essential link between extracellular matrix proteins, like laminin, and the cellular cytoskeleton via the dystrophin-glycoprotein complex. In secondary dystroglycanopathy muscular dystrophy, glycosylation abnormalities disrupt a complex O-mannose glycan necessary for muscle structural integrity and signaling. Fktn-deficient dystroglycanopathy mice develop moderate to severe...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Identifiers

PMID: 26751696
DOI: 10.1371/journal.pone.0147049
PMCID: PMC4708996

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