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SMN expression is required in motor neurons to rescue electrophysiological deficits in the SMNΔ7 mouse model of SMA

Proximal spinal muscular atrophy (SMA) is the most frequent cause of hereditary infant mortality. SMA is an autosomal recessive neuromuscular disorder that results from the loss of the Survival Motor Neuron 1 (SMN1) gene and retention of the SMN2 gene. The SMN2 gene produces an insufficient amount o...

詳細記述

保存先:
書誌詳細
出版年:Hum Mol Genet
主要な著者: McGovern, Vicki L., Iyer, Chitra C., Arnold, W. David, Gombash, Sara E., Zaworski, Phillip G., Blatnik, Anton J., Foust, Kevin D., Burghes, Arthur H.M.
フォーマット: Artigo
言語:Inglês
出版事項: Oxford University Press 2015
主題:
オンライン・アクセス:https://ncbi.nlm.nih.gov/pmc/articles/PMC4572068/
https://ncbi.nlm.nih.gov/pubmed/26206889
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1093/hmg/ddv283
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