PUBLICATION

Knockdown of the survival motor neuron (Smn) protein in zebrafish causes defects in motor axon outgrowth and pathfinding

Authors
McWhorter, M.L., Monani, U.R., Burghes, A.H., and Beattie, C.E.
ID
ZDB-PUB-030908-5
Date
2003
Source
The Journal of cell biology   162(5): 919-932 (Journal)
Registered Authors
Beattie, Christine
Keywords
none
MeSH Terms
  • Animals
  • Axons/metabolism*
  • Cell Death/physiology
  • Cell Movement/physiology*
  • Cyclic AMP Response Element-Binding Protein
  • Humans
  • In Situ Hybridization
  • Motor Neurons/cytology
  • Motor Neurons/metabolism*
  • Muscular Atrophy, Spinal/metabolism
  • Nerve Tissue Proteins/genetics
  • Nerve Tissue Proteins/metabolism*
  • Neuromuscular Junction
  • Oligonucleotides, Antisense/metabolism
  • RNA-Binding Proteins
  • Receptors, Cholinergic/metabolism
  • SMN Complex Proteins
  • Survival of Motor Neuron 1 Protein
  • Zebrafish/anatomy & histology
  • Zebrafish/embryology*
  • Zebrafish/physiology
PubMed
12952942 Full text @ J. Cell Biol.
Abstract
Spinal muscular atrophy (SMA) is an autosomal recessive disorder characterized by a loss of alpha motoneurons in the spinal cord. SMA is caused by low levels of the ubiquitously expressed survival motor neuron (Smn) protein. As it is unclear how low levels of Smn specifically affect motoneurons, we have modeled SMA in zebrafish, a vertebrate model organism with well-characterized motoneuron development. Using antisense morpholinos to reduce Smn levels throughout the entire embryo, we found motor axon-specific pathfinding defects. Reduction of Smn in individual motoneurons revealed that smn is acting cell autonomously. These results show for the first time, in vivo, that Smn functions in motor axon development and suggest that these early developmental defects may lead to subsequent motoneuron loss.
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