Distinct Fiber Type Signature in Mouse Muscles Expressing a Mutant Lamin A Responsible for Congenital Muscular Dystrophy in a Patient

Distinct Fiber Type Signature in Mouse Muscles Expressing a Mutant Lamin A Responsible for Congenital Muscular Dystrophy in a Patient

Specific mutations in LMNA, which encodes nuclear intermediate filament proteins lamins A/C, affect skeletal muscle tissues. Early-onset LMNA myopathies reveal different alterations of muscle fibers, including fiber type disproportion or prominent dystrophic and/or inflammatory changes. Recently, we...

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Bibliographic Details
Main Authors: Alice Barateau, Nathalie Vadrot, Onnik Agbulut, Patrick Vicart, Sabrina Batonnet-Pichon, Brigitte Buendia
Format: Article
Language:English
Published: MDPI AG 2017-04-01
Series:Cells
Subjects:
lamin A
congenital muscular dystrophy
muscle fiber type transition
myosin heavy chain IIA
Online Access:http://www.mdpi.com/2073-4409/6/2/10

Internet

http://www.mdpi.com/2073-4409/6/2/10

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