Fishing for a mechanism: using zebrafish to understand spinal muscular atrophy

Christine E Beattie, Tessa L Carrel, Michelle L McWhorter
Journal of Child Neurology 2007, 22 (8): 995-1003
Motoneuron 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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