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KMID : 1161520140180050311
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Animal Cells and Systems
2014 Volume.18 No. 5 p.311 ~ p.317
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Adenoviral-mediated mouse model of motor impairment in distal spinal muscular atrophy type V
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Seo Ah-Jung
Park Byung-Sun
Jeong Na-Young
Kim Do-Yen
Kim Sung-Hoon
Park Chan
Jung Jun-Yang
Huh Young-Buhm
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Abstract
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Distal spinal muscular atrophy type V (dSMA-V) is a hereditary neurodegenerative axonal neuropathy and a glycyl-tRNA synthetase (GARS)-associated neuropathy that is caused by a mutation in GARS. Herein, we report a new GARS-associated neuropathy mouse model using an adenovirus vector system equipped with a neuron-specific promoter. In this model, we showed an increased number of activated microglia around the L129P mutant-expressing motor neuron cell bodies and the increased nerve injury signal in L129P mutant-expressing motor neuron cell bodies. In addition, we found that the mouse model exhibited impaired motor function caused by the L129P mutant. We propose that the neuroinflammation may be associated with motor neuron impairment in GARS-associated neuropathies.
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KEYWORD
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recombinant adenovirus, glycyl-tRNA synthetase, motor impairment, ATF3, microglia
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