期刊
JOURNAL OF CHILD NEUROLOGY
卷 22, 期 8, 页码 995-1003出版社
SAGE PUBLICATIONS INC
DOI: 10.1177/0883073807305671
关键词
spinal muscular atrophy; SMN; zebrafish
资金
- NINDS NIH HHS [R01NS050414, P30-NS045758] Funding Source: Medline
Motoncuron diseases cause paralysis and death due to loss of motoneurons that innervate skeletal muscle. Spinal muscular atrophy is a human motoneuron disease that is genetically linked to the survival motor neuron gene (SMN). Although SMN was identified more than a decade ago, it remains unclear how decreased levels of the SMN protein cause spinal muscular atrophy. The use of animal models, however, offers a crucial tool in determining the function of SMN in this disease. In this review, we discuss our efforts to develop a zebrafish model of spinal muscular atrophy.
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