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...
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: | |
Online Access: | http://www.mdpi.com/2073-4409/6/2/10 |
Similar Items
-
Lamin A/C Assembly Defects in <i>LMNA</i>-Congenital Muscular Dystrophy Is Responsible for the Increased Severity of the Disease Compared with Emery–Dreifuss Muscular Dystrophy
by: Anne T. Bertrand, et al.
Published: (2020-03-01) -
Muscular Dystrophy /
by: Naff, Clay Farris, editor 192865, et al.
Published: (2012) -
A rare case of dystrophinopathy: Duchenne muscular dystrophy–Becker muscular dystrophy intermediate complex
by: Rachna Gulati, et al.
Published: (2022-01-01) -
Mass Spectrometric Profiling of Extraocular Muscle and Proteomic Adaptations in the <i>mdx-4cv</i> Model of Duchenne Muscular Dystrophy
by: Stephen Gargan, et al.
Published: (2021-06-01) -
Congenital muscular dystrophy a case study with a mutation in the POMT1 gene
by: Muhsin Elmas, et al.
Published: (2019-12-01)