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

Puttaparthi K, Van Kaer L, Elliott JL
Exp Neurol. 2007 206 (1): 53-8

PMID: 17482163 · PMCID: PMC2692686 · DOI:10.1016/j.expneurol.2007.03.024

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.

MeSH Terms (17)

Amyotrophic Lateral Sclerosis Animals Cysteine Endopeptidases Disease Models, Animal Disease Progression Humans Mice Mice, Knockout Mice, Transgenic Motor Neurons Mutation Proteasome Endopeptidase Complex Spinal Cord Superoxide Dismutase Superoxide Dismutase-1 Survival Rate Up-Regulation

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