Beattie, C.E., Carrel, T.L., and McWhorter, M.L. (2007) Fishing for a mechanism: using zebrafish to understand spinal muscular atrophy. Journal of Child Neurology. 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.