Advances in the understanding of skeletal muscle weakness in murine models of diseases affecting nerve-evoked muscle activity, motor neurons, synapses and myofibers

Highlights • Impaired maximal activation capacity is not highly related to Hdac4 gene upregulation. • Muscle atrophy only explains 37%, 27% and 0% of weakness in SOD1G93A , Neurotomized and Musk mice. • Our results suggest LC3 , Fn14 , Bcl3 and Gadd45a as candidate genes involved in the maintenance...

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Published in:Neuromuscular disorders : NMD 2014-11, Vol.24 (11), p.960-972
Main Authors: Ferry, Arnaud, Joanne, Pierre, Hadj-Said, Wahiba, Vignaud, Alban, Lilienbaum, Alain, Hourdé, Christophe, Medja, Fadia, Noirez, Philippe, Charbonnier, Frederic, Chatonnet, Arnaud, Chevessier, Frederic, Nicole, Sophie, Agbulut, Onnik, Butler-Browne, Gillian
Format: Article
Language:English
Subjects:
Animals
Cellular Biology
Cholinesterases - genetics
Disease Models, Animal
Electromyography
Evoked Potentials, Motor - genetics
Evoked Potentials, Motor - physiology
Gene Expression Regulation - genetics
Heparan Sulfate Proteoglycans - genetics
Life Sciences
Mice
Mice, Transgenic
Motor Neurons - pathology
Murine models
Muscle Weakness - etiology
Muscle, Skeletal - physiopathology
Muscular Diseases - complications
Muscular Diseases - genetics
Mutation - genetics
Myofibrils - pathology
Neuroeffector Junction - physiopathology
Neurology
Neuromuscular diseases
Receptor Protein-Tyrosine Kinases - genetics
Receptors, Tumor Necrosis Factor - genetics
Skeletal muscle weakness
Superoxide Dismutase - genetics
TWEAK Receptor
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Summary:Highlights • Impaired maximal activation capacity is not highly related to Hdac4 gene upregulation. • Muscle atrophy only explains 37%, 27% and 0% of weakness in SOD1G93A , Neurotomized and Musk mice. • Our results suggest LC3 , Fn14 , Bcl3 and Gadd45a as candidate genes involved in the maintenance of the severe atrophic state. • In conclusion, our study indicates that muscle weakness can result from the triggering of different signaling pathways.
ISSN:0960-8966
1873-2364
DOI:10.1016/j.nmd.2014.06.001