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Article

Intracellular spatially-targeted chemical chaperones increase native state stability of mutant SOD1 barrel.

Biological chemistry - 26 Sept 2023

Ribeiro Sara S, Gnutt David, Azoulay-Ginsburg Salome, Fetahaj Zamira, Spurlock Ella, Lindner Felix, Kuz Damon, Cohen-Erez Yfat, Rapaport Hanna, Israelson Adrian, Gruzman Arie-Lev, Ebbinghaus Simon

Abstract excerpt

Amyotrophic lateral sclerosis (ALS) is a progressive neurological disorder with currently no cure. Central to the cellular dysfunction associated with this fatal proteinopathy is the accumulation of unfolded/misfolded superoxide dismutase 1 (SOD1) in various subcellular locations. The molecular mechanism driving the formation of SOD1 aggregates is not fully understood but numerous studies suggest that aberrant...

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PMID: 37555646
DOI: 10.1515/hsz-2023-0198

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