Abstract
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.
Publication types
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Research Support, N.I.H., Extramural
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Research Support, Non-U.S. Gov't
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Review
MeSH terms
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Animals
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Cell Differentiation / genetics
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Cyclic AMP Response Element-Binding Protein / genetics*
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Cyclic AMP Response Element-Binding Protein / metabolism
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Disease Models, Animal
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Genetic Predisposition to Disease / genetics*
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Humans
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Motor Neurons / metabolism
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Motor Neurons / pathology
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Muscle, Skeletal / embryology
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Muscle, Skeletal / innervation
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Muscle, Skeletal / physiopathology
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Muscular Atrophy, Spinal / genetics*
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Muscular Atrophy, Spinal / metabolism*
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Muscular Atrophy, Spinal / physiopathology
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Mutation / genetics
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Nerve Tissue Proteins / genetics*
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Nerve Tissue Proteins / metabolism
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RNA-Binding Proteins / genetics*
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RNA-Binding Proteins / metabolism
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SMN Complex Proteins
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Survival of Motor Neuron 1 Protein
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Zebrafish / embryology*
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Zebrafish / genetics*
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Zebrafish / metabolism
Substances
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Cyclic AMP Response Element-Binding Protein
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Nerve Tissue Proteins
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RNA-Binding Proteins
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SMN Complex Proteins
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Survival of Motor Neuron 1 Protein
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smn1 protein, zebrafish