Assessing the role of immuno-proteasomes in a mouse model of familial ALS

Krishna Puttaparthi, Luc Van Kaer, Jeffrey L. Elliott

Research output: Contribution to journalArticlepeer-review

17 Scopus citations


The accumulation of protein aggregates is thought to be an important component in the pathogenesis of mutant SOD1-induced disease. Mutant SOD1 aggregates appear to be cleared by proteasomes, at least in vitro, suggesting a potentially important role for proteasome degradation pathways in vivo. G93A SOD1 transgenic mice show an increase in proteasome activity and induction of immuno-proteasome subunits within spinal cord as they develop neurological symptoms. To determine what role immuno-proteasomes may have in mutant SOD1-induced disease, we crossed G93A SOD1 transgenic mice with LMP2-/- mice to obtain G93A SOD1 mice lacking the LMP2 immuno-proteasome subunit. G93A SOD1/LMP2-/- mice show significant reductions in proteasome function within spinal cord compared to G93A SOD1 mice. However, G93A SOD1/LMP2-/- mice show no change in motor function decline, or survival compared to G93A SOD1 mice. These results indicate that the loss of immuno-proteasome function in vivo does not significantly alter mutant SOD1-induced disease.

Original languageEnglish (US)
Pages (from-to)53-58
Number of pages6
JournalExperimental Neurology
Issue number1
StatePublished - Jul 2007


  • ALS
  • Aggregation
  • Astrocyte
  • Microglia
  • Motor neuron
  • Spinal cord

ASJC Scopus subject areas

  • Neurology
  • Developmental Neuroscience


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