Article
Potential effect of S-nitrosylated protein disulfide isomerase on mutant SOD1 aggregation and neuronal cell death in amyotrophic lateral sclerosis.
Molecular neurobiology - 1 Apr 2014
Jeon Gye Sun, Nakamura Tomohiro, Lee Jeong-Seon, Choi Won-Jun, Ahn Suk-Won, Lee Kwang-Woo, Sung Jung-Joon, Lipton Stuart A
Abstract excerpt
Aggregation of misfolded protein and resultant intracellular inclusion body formation are common hallmarks of mutant superoxide dismutase (mSOD1)-linked familial amyotrophic lateral sclerosis (FALS) and have been associated with the selective neuronal death. Protein disulfide isomerase (PDI) represents a family of enzymatic chaperones that can fold nascent and aberrant proteins in the endoplasmic reticulum (ER)...
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