Soma size and Cav1.3 channel expression in vulnerable and resistant motoneuron populations of the SOD1G93A mouse model of ALS

Liza Shoenfeld, Ruth E. Westenbroek, Erika Fisher, Katharina A. Quinlan, Vicki M. Tysseling, Randall K. Powers, Charles J. Heckman, Marc D. Binder*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

8 Scopus citations


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 SOD1G93A mice exhibit an enlargement of soma size (i.e., cross-sectional area) and an increase in Cav1.3 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 SOD1G93A 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 Cav1.3 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.

Original languageEnglish (US)
Article numbere12113
JournalPhysiological reports
Issue number8
StatePublished - 2014


  • ALS
  • Ca1.3 channels
  • Motoneuron size
  • Motoneurons in SOD1 mice

ASJC Scopus subject areas

  • Physiology
  • Physiology (medical)

Fingerprint Dive into the research topics of 'Soma size and Ca<sub>v</sub>1.3 channel expression in vulnerable and resistant motoneuron populations of the SOD1<sup>G93A</sup> mouse model of ALS'. Together they form a unique fingerprint.

Cite this