SMAD6 overexpression leads to accelerated myogenic differentiation of LMNA mutated cells.

ABSTRACT: LMNA gene encodes lamins A and C, two major components of the nuclear lamina, a network of intermediate filaments underlying the inner nuclear membrane. Most of LMNA mutations are associated with cardiac and/or skeletal muscles defects. Muscle laminopathies include Emery-Dreifuss Muscular Dystrophy, Limb-Girdle Muscular Dystrophy 1B, LMNA-related Congenital Muscular Dystrophy and Dilated Cardiomyopathy with conduction defects. To identify potential alterations in signaling pathways regulating muscle differentiation in LMNA-mutated myoblasts, we used a previously described model of conditionally immortalized murine myoblasts: H-2K cell lines. Comparing gene expression profiles in wild-type and Lmna^∆8-11 H-2K myoblasts, we identified two major alterations in the BMP (Bone Morphoge

SUBMITTER: Janin A 

PROVIDER: S-EPMC5884786 | biostudies-literature | 2018 Apr

REPOSITORIES: biostudies-literature