Nuclear changes in skeletal muscle extend to satellite cells in autosomal dominant Emery-Dreifuss muscular dystrophy/limb-girdle muscular dystrophy 1B

YE Park, YK Hayashi, K Goto, H Komaki… - Neuromuscular …, 2009 - Elsevier
Autosomal forms of Emery-Dreifuss muscular dystrophy (AD-/AR-EDMD) and limb-girdle
muscular dystrophy type 1B (LGMD1B) are caused by mutations in the gene encoding A-
type lamins (LMNA). A-type lamins are major components of nuclear lamina and known to
have important roles in maintaining nuclear integrity. LMNA mutations are also suggested to
cause reduced myogenic differentiation potentials, implying that satellite cell nuclei in AD-
EDMD/LGMD1B are likewise affected. We examined nuclear changes of skeletal muscles …