Article
Effect of CCS on the accumulation of FALS SOD1 mutant-containing aggregates and on mitochondrial translocation of SOD1 mutants: implication of a free radical hypothesis.
Archives of biochemistry and biophysics - 15 May 2011
Kim Ha Kun, Chung Youn Wook, Chock P Boon, Yim Moon B
Abstract excerpt
Missense mutations of SOD1 are linked to familial amyotrophic lateral sclerosis (FALS) through a yet-to-be identified toxic-gain-of-function. One of the proposed mechanisms involves enhanced aggregate formation. However, a recent study showed that dual transgenic mice overexpressing both G93A and CCS copper chaperone (G93A/CCS) exhibit no SOD1-positive aggregates yet show accelerated FALS symptoms with enhanced...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.