Severe neuromuscular denervation of clinically relevant muscles in a mouse model of spinal muscular atrophy

Spinal muscular atrophy (SMA), a motoneuron disease caused by a deficiency of the survival of motor neuron (SMN) protein, is characterized by motoneuron loss and muscle weakness. It remains unclear whether widespread loss of neuromuscular junctions (NMJs) is involved in SMA pathogenesis. We undertoo...

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Veröffentlicht in:	Human molecular genetics 2012-01, Vol.21 (1), p.185-195
Hauptverfasser:	Ling, Karen K. Y., Gibbs, Rebecca M., Feng, Zhihua, Ko, Chien-Ping
Format:	Artikel
Sprache:	eng
Schlagworte:	Animal models Animals Biological and medical sciences Cell survival Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases Denervation Disease Models, Animal Fundamental and applied biological sciences. Psychology Genetics of eukaryotes. Biological and molecular evolution Innervation Male Medical sciences Mice Mice, Knockout Mice, Transgenic Molecular and cellular biology Motor neurons Muscle Denervation Muscle, Skeletal - innervation Muscle, Skeletal - pathology Muscle, Skeletal - surgery Muscles Muscular Atrophy, Spinal - metabolism Muscular Atrophy, Spinal - pathology Muscular Atrophy, Spinal - surgery Nerve Degeneration Neurofilaments Neurology Neuromuscular Junction - metabolism Neuromuscular Junction - surgery Neuromuscular junctions SMN protein spinal muscular atrophy Synapses Synapses - metabolism Synapses - pathology Trichostatin A
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Zusammenfassung:	Spinal muscular atrophy (SMA), a motoneuron disease caused by a deficiency of the survival of motor neuron (SMN) protein, is characterized by motoneuron loss and muscle weakness. It remains unclear whether widespread loss of neuromuscular junctions (NMJs) is involved in SMA pathogenesis. We undertook a systematic examination of NMJ innervation patterns in >20 muscles in the SMNΔ7 SMA mouse model. We found that severe denervation (
ISSN:	0964-6906 1460-2083
DOI:	10.1093/hmg/ddr453