PUBLICATION

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

Authors
Beattie, C.E., Carrel, T.L., and McWhorter, M.L.
ID
ZDB-PUB-070907-23
Date
2007
Source
Journal of Child Neurology   22(8): 995-1003 (Review)
Registered Authors
Beattie, Christine, Carrel, Tessa, McWhorter, Michelle
Keywords
spinal muscular atrophy, SMN, zebrafish
MeSH Terms
  • Animals
  • Cell Differentiation/genetics
  • Cyclic AMP Response Element-Binding Protein/genetics*
  • Cyclic AMP Response Element-Binding Protein/metabolism
  • Disease Models, Animal
  • Genetic Predisposition to Disease/genetics*
  • Humans
  • Motor Neurons/metabolism
  • Motor Neurons/pathology
  • Muscle, Skeletal/embryology
  • Muscle, Skeletal/innervation
  • Muscle, Skeletal/physiopathology
  • Muscular Atrophy, Spinal/genetics*
  • Muscular Atrophy, Spinal/metabolism*
  • Muscular Atrophy, Spinal/physiopathology
  • Mutation/genetics
  • Nerve Tissue Proteins/genetics*
  • Nerve Tissue Proteins/metabolism
  • RNA-Binding Proteins/genetics*
  • RNA-Binding Proteins/metabolism
  • SMN Complex Proteins
  • Survival of Motor Neuron 1 Protein
  • Zebrafish/embryology*
  • Zebrafish/genetics*
  • Zebrafish/metabolism
PubMed
17761655 Full text @ J. Child Neurol.
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.
Genes / Markers
Figures
Expression
Phenotype
Mutation and Transgenics
Human Disease / Model Data
Sequence Targeting Reagents
Fish
Antibodies
Orthology
Engineered Foreign Genes
Mapping
Errata and Notes