期刊
AMYOTROPHIC LATERAL SCLEROSIS
卷 13, 期 4, 页码 333-340出版社
INFORMA HEALTHCARE
DOI: 10.3109/17482968.2012.648645
关键词
ALS; mitochondria; misfolded SOD1; antibodies; motor neuron
资金
- ALS Society of Canada
- CIHR
- CFI
- CRCHUM
- FRSQ
- MDA
Mutations in SOD1, causative for a subset of familial ALS cases, are associated with the formation of non-normal SOD1 conformers. Recent studies have defined this pool of SOD1 as misfolded and new antibodies have been developed to selectively detect misfolded SOD1 in vivo and in vitro. We will review these new tools and expand on the evidence demonstrating mitochondria as a common intersecting point for misfolded SOD1.
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