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Soma size and Ca(v)1.3 channel expression in vulnerable and resistant motoneuron populations of the SOD1(G93A) mouse model of ALS
Although the loss of motoneurons is an undisputed feature of amyotrophic lateral sclerosis (ALS) in man and in its animal models (SOD1 mutant mice), how the disease affects the size and excitability of motoneurons prior to their degeneration is not well understood. This study was designed to test th...
Autores principales: | , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Wiley Periodicals, Inc.
2014
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4246589/ https://www.ncbi.nlm.nih.gov/pubmed/25107988 http://dx.doi.org/10.14814/phy2.12113 |
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author | Shoenfeld, Liza Westenbroek, Ruth E. Fisher, Erika Quinlan, Katharina A. Tysseling, Vicki M. Powers, Randall K. Heckman, Charles J. Binder, Marc D. |
author_facet | Shoenfeld, Liza Westenbroek, Ruth E. Fisher, Erika Quinlan, Katharina A. Tysseling, Vicki M. Powers, Randall K. Heckman, Charles J. Binder, Marc D. |
author_sort | Shoenfeld, Liza |
collection | PubMed |
description | Although the loss of motoneurons is an undisputed feature of amyotrophic lateral sclerosis (ALS) in man and in its animal models (SOD1 mutant mice), how the disease affects the size and excitability of motoneurons prior to their degeneration is not well understood. This study was designed to test the hypothesis that motoneurons in mutant SOD1(G93A) mice exhibit an enlargement of soma size (i.e., cross‐sectional area) and an increase in Ca(v)1.3 channel expression at postnatal day 30, well before the manifestation of physiological symptoms that typically occur at p90 (Chiu et al. 1995). We made measurements of spinal and hypoglossal motoneurons vulnerable to degeneration, as well as motoneurons in the oculomotor nucleus that are resistant to degeneration. Overall, we found that the somata of motoneurons in male SOD1(G93A) mutants were larger than those in wild‐type transgenic males. When females were included in the two groups, significance was lost. Expression levels of the Ca(v)1.3 channels were not differentiated by genotype, sex, or any interaction of the two. These results raise the intriguing possibility of an interaction between male sex steroid hormones and the SOD1 mutation in the etiopathogenesis of ALS. |
format | Online Article Text |
id | pubmed-4246589 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2014 |
publisher | Wiley Periodicals, Inc. |
record_format | MEDLINE/PubMed |
spelling | pubmed-42465892014-12-18 Soma size and Ca(v)1.3 channel expression in vulnerable and resistant motoneuron populations of the SOD1(G93A) mouse model of ALS Shoenfeld, Liza Westenbroek, Ruth E. Fisher, Erika Quinlan, Katharina A. Tysseling, Vicki M. Powers, Randall K. Heckman, Charles J. Binder, Marc D. Physiol Rep Original Research Although the loss of motoneurons is an undisputed feature of amyotrophic lateral sclerosis (ALS) in man and in its animal models (SOD1 mutant mice), how the disease affects the size and excitability of motoneurons prior to their degeneration is not well understood. This study was designed to test the hypothesis that motoneurons in mutant SOD1(G93A) mice exhibit an enlargement of soma size (i.e., cross‐sectional area) and an increase in Ca(v)1.3 channel expression at postnatal day 30, well before the manifestation of physiological symptoms that typically occur at p90 (Chiu et al. 1995). We made measurements of spinal and hypoglossal motoneurons vulnerable to degeneration, as well as motoneurons in the oculomotor nucleus that are resistant to degeneration. Overall, we found that the somata of motoneurons in male SOD1(G93A) mutants were larger than those in wild‐type transgenic males. When females were included in the two groups, significance was lost. Expression levels of the Ca(v)1.3 channels were not differentiated by genotype, sex, or any interaction of the two. These results raise the intriguing possibility of an interaction between male sex steroid hormones and the SOD1 mutation in the etiopathogenesis of ALS. Wiley Periodicals, Inc. 2014-08-08 /pmc/articles/PMC4246589/ /pubmed/25107988 http://dx.doi.org/10.14814/phy2.12113 Text en © 2014 The Authors. Physiological Reports published by Wiley Periodicals, Inc. on behalf of the American Physiological Society and The Physiological Society. http://creativecommons.org/licenses/by/3.0/ This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Original Research Shoenfeld, Liza Westenbroek, Ruth E. Fisher, Erika Quinlan, Katharina A. Tysseling, Vicki M. Powers, Randall K. Heckman, Charles J. Binder, Marc D. Soma size and Ca(v)1.3 channel expression in vulnerable and resistant motoneuron populations of the SOD1(G93A) mouse model of ALS |
title | Soma size and Ca(v)1.3 channel expression in vulnerable and resistant motoneuron populations of the SOD1(G93A) mouse model of ALS |
title_full | Soma size and Ca(v)1.3 channel expression in vulnerable and resistant motoneuron populations of the SOD1(G93A) mouse model of ALS |
title_fullStr | Soma size and Ca(v)1.3 channel expression in vulnerable and resistant motoneuron populations of the SOD1(G93A) mouse model of ALS |
title_full_unstemmed | Soma size and Ca(v)1.3 channel expression in vulnerable and resistant motoneuron populations of the SOD1(G93A) mouse model of ALS |
title_short | Soma size and Ca(v)1.3 channel expression in vulnerable and resistant motoneuron populations of the SOD1(G93A) mouse model of ALS |
title_sort | soma size and ca(v)1.3 channel expression in vulnerable and resistant motoneuron populations of the sod1(g93a) mouse model of als |
topic | Original Research |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4246589/ https://www.ncbi.nlm.nih.gov/pubmed/25107988 http://dx.doi.org/10.14814/phy2.12113 |
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