Premium
Alterations in the myogenic capacity of satellite cells in a mouse model of ALS
Author(s) -
English Samuel Andrew,
Gupta Sumeet,
Clermont Daphney,
Chen Dapeng,
Mazala Davi,
Chin Eva R
Publication year - 2012
Publication title -
the faseb journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.709
H-Index - 277
eISSN - 1530-6860
pISSN - 0892-6638
DOI - 10.1096/fasebj.26.1_supplement.1078.28
Subject(s) - myogenesis , amyotrophic lateral sclerosis , atrophy , population , satellite , skeletal muscle , muscle atrophy , biology , medicine , myocyte , endocrinology , wild type , microbiology and biotechnology , mutant , disease , genetics , environmental health , aerospace engineering , gene , engineering
Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig's disease, is an invariably fatal progressive neurodegenerative disorder that results in pervasive muscle wasting. The purpose of this study was to characterize a possible defect in the myogenic capacity of the skeletal muscle resident satellite cell population in the SOD1 G93A ALS mouse. Satellite cells were obtained from the flexor digitorum brevis (FDB) muscle of wild‐type control (CON) and SOD1 G93A (ALS) mice and allowed to differentiate into cultured myotubes. In 90d old pre‐symptomatic mice, we observed significant decreases in the number of nuclei per myotube (4.97 ± 1.7 vs. 2.48 ± 1.1, p=0.051; CON vs. ALS), number of myotubes (5.58 ± 0.37 vs. 3.39 ± 1.7, p=0.049) and fusion index (% of total nuclei fused into myotubes; 32.1 ± 3.6 vs. 17.4 ± 5.4, p<0.01) in cultures from wild‐type CON vs. SOD1 G93A ALS. These data suggest that the myogenic capacity of the satellite cell population in a mutant mouse model of ALS is impaired in a pre‐symptomatic stage of the disease, and thus may contribute to the ongoing atrophy process.