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Human Molecular Genetics Advance Access published online on September 28, 2005
Human Molecular Genetics, doi:10.1093/hmg/ddi357
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© The Author 2005. Published by Oxford University Press. All rights reserved. The online version of this article has been published under an open access model. Users are entitled to use, reproduce, disseminate, or display the open access version of this article for non-commercial purposes provided that: the original authorship is properly and fully attributed; the Journal and Oxford University Press are attributed as the original place of publication with the correct citation details given; if an article is subsequently reproduced or disseminated not in its entirety but only in part or as a derivative work this must be clearly indicated. For commercial re-use, please contact journals.permissions@oupjournals.org
Received August 25, 2005
Revised September 23, 2005
Accepted September 23, 2005

Article

No widespread induction of cell death genes occurs in pure motoneurons in an ALS mouse model

Florence E. Perrin 1, Gaelle Boisset 1, Mylene Docquier 2, Olivier Schaad 2, Patrick Descombes 2, and Ann C. Kato 3*

1 Department of Basic Neuroscience, Faculty of Medicine, National Center of Competence in Research "Frontiers in Genetics", 1211 Geneva 4, Switzerland
2 Genomics Platform, National Center of Competence in Research "Frontiers in Genetics", 1211 Geneva 4, Switzerland
3 Department of Basic Neuroscience, Centre Médical Universitaire, 1 rue Michel Servet, 1211 Geneva 4, Switzerland

* To whom correspondence should be addressed.
Ann C. Kato, E-mail: Ann.Kato{at}medecine.unige.ch


  Abstract

To identify candidate genes that may be involved in motoneuron degeneration, we combined laser capture microdissection with microarray technology. Gene expression in motoneurons was analyzed during the progression of the disease in transgenic SOD1G93A mice that develop motoneuron loss. Three major observations were made : first, there was only a small number of genes that were differentially expressed in motoneurons at a pre-symptomatic age (27 out of 34,000 transcripts). Secondly, there is an early specific up-regulation of the gene coding for the intermediate filament vimentin that is increased even further during disease progression. Using in situ hybridization and immunohistochemical analysis, we show that vimentin expression was not only elevated in motoneurons but that the protein formed inclusions in the motoneuron cytoplasm. Thirdly, a time-course analysis of the motoneurons at a symptomatic age (90 and 120 days) showed a modest de-regulation of only a few genes associated with cell death pathways; however, a massive up-regulation of genes involved in cell growth and/or maintenance was observed. This is the first description of the gene profile of SOD1G93A motoneurons during disease progression and unexpectedly, no widespread induction of cell death-associated genes was detected in motoneurons of SOD1G93A mice.


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