Reference - Detail
|Author||McWhorter ML, Monani UR, Burghes AH, Beattie CE.|
|Title||Knockdown of the survival motor neuron (Smn) protein in zebrafish causes defects in motor axon outgrowth and pathfinding.|
|Journal||J. Cell Biol.|
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.
|MeSH||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|
|WOS Category||CELL BIOLOGY|