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Journal of Child Neurology, Vol. 22, No. 8, 995-1003 (2007)
DOI: 10.1177/0883073807305671

Fishing for a Mechanism: Using Zebrafish to Understand Spinal Muscular Atrophy

Christine E. Beattie, PhD

Ohio State University Center for Molecular Neurobiology and Department of Neuroscience, Columbus, beattie.24{at}osu.edu

Tessa L. Carrel, PhD

Ohio State University Center for Molecular Neurobiology and Department of Neuroscience, Columbus

Michelle L. McWhorter, PhD

Ohio State University Center for Molecular Neurobiology and Department of Neuroscience, Columbus

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.

Key Words: spinal muscular atrophy • SMN • zebrafish


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