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TAR DNA‐binding protein 43 (TDP‐43) is an RNA‐binding protein and a major component of protein aggregates found in amyotrophic lateral sclerosis and several other neurodegenerative diseases. TDP‐43 exists as a full‐length protein and as two shorter forms of 25 and 35 kDa. Full‐length mutant TDP‐43s found in amyotrophic lateral sclerosis patients re‐localize from the nucleus to the cytoplasm and in...
J. Neurochem. (2010) 112, 183–192.
AbstractThe copper‐enzyme cytochrome c oxidase (Cytox) has been indicated as a primary molecular target of mutant copper, zinc superoxide dismutase (SOD1) in familial amyotrophic lateral sclerosis (fALS); however, the mechanism underlying its inactivation is still unclear. As the toxicity of mutant SOD1s could arise from their selective recruitment to mitochondria,...
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