Ontology highlight
ABSTRACT:
SUBMITTER: Jackrel ME
PROVIDER: S-EPMC3909490 | biostudies-literature | 2014 Jan
REPOSITORIES: biostudies-literature
Jackrel Meredith E ME DeSantis Morgan E ME Martinez Bryan A BA Castellano Laura M LM Stewart Rachel M RM Caldwell Kim A KA Caldwell Guy A GA Shorter James J
Cell 20140101 1-2
There are no therapies that reverse the proteotoxic misfolding events that underpin fatal neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS) and Parkinson's disease (PD). Hsp104, a conserved hexameric AAA+ protein from yeast, solubilizes disordered aggregates and amyloid but has no metazoan homolog and only limited activity against human neurodegenerative disease proteins. Here, we reprogram Hsp104 to rescue TDP-43, FUS, and α-synuclein proteotoxicity by mutating single re ...[more]