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

Spinal muscular atrophy (SMA) is an autosomal recessive disorder characterized by a loss of α 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...

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Detalles Bibliográficos
Autores principales: McWhorter, Michelle L., Monani, Umrao R., Burghes, Arthur H.M., Beattie, Christine E.
Formato: Texto
Lenguaje:English
Publicado: The Rockefeller University Press 2003
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1761110/
https://www.ncbi.nlm.nih.gov/pubmed/12952942
http://dx.doi.org/10.1083/jcb.200303168
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author McWhorter, Michelle L.
Monani, Umrao R.
Burghes, Arthur H.M.
Beattie, Christine E.
author_facet McWhorter, Michelle L.
Monani, Umrao R.
Burghes, Arthur H.M.
Beattie, Christine E.
author_sort McWhorter, Michelle L.
collection PubMed
description Spinal muscular atrophy (SMA) is an autosomal recessive disorder characterized by a loss of α 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.
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spelling pubmed-17611102008-05-01 Knockdown of the survival motor neuron (Smn) protein in zebrafish causes defects in motor axon outgrowth and pathfinding McWhorter, Michelle L. Monani, Umrao R. Burghes, Arthur H.M. Beattie, Christine E. J Cell Biol Article Spinal muscular atrophy (SMA) is an autosomal recessive disorder characterized by a loss of α 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. The Rockefeller University Press 2003-09-01 /pmc/articles/PMC1761110/ /pubmed/12952942 http://dx.doi.org/10.1083/jcb.200303168 Text en Copyright © 2003, The Rockefeller University Press This article is distributed under the terms of an Attribution–Noncommercial–Share Alike–No Mirror Sites license for the first six months after the publication date (see http://www.rupress.org/terms). After six months it is available under a Creative Commons License (Attribution–Noncommercial–Share Alike 4.0 Unported license, as described at http://creativecommons.org/licenses/by-nc-sa/4.0/).
spellingShingle Article
McWhorter, Michelle L.
Monani, Umrao R.
Burghes, Arthur H.M.
Beattie, Christine E.
Knockdown of the survival motor neuron (Smn) protein in zebrafish causes defects in motor axon outgrowth and pathfinding
title Knockdown of the survival motor neuron (Smn) protein in zebrafish causes defects in motor axon outgrowth and pathfinding
title_full Knockdown of the survival motor neuron (Smn) protein in zebrafish causes defects in motor axon outgrowth and pathfinding
title_fullStr Knockdown of the survival motor neuron (Smn) protein in zebrafish causes defects in motor axon outgrowth and pathfinding
title_full_unstemmed Knockdown of the survival motor neuron (Smn) protein in zebrafish causes defects in motor axon outgrowth and pathfinding
title_short Knockdown of the survival motor neuron (Smn) protein in zebrafish causes defects in motor axon outgrowth and pathfinding
title_sort knockdown of the survival motor neuron (smn) protein in zebrafish causes defects in motor axon outgrowth and pathfinding
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1761110/
https://www.ncbi.nlm.nih.gov/pubmed/12952942
http://dx.doi.org/10.1083/jcb.200303168
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