Literature associating DNAJB6 and autosomal dominant limb-girdle muscular dystrophy type 1H
DnaJ (Hsp40) homolog, subfamily B, member 6; Plays an indispensable role in the organization of KRT8/KRT18 filaments. Acts as an endogenous molecular chaperone for neuronal proteins including huntingtin. Suppresses aggregation and toxicity of polyglutamine-containing, aggregation-prone proteins. Isoform B but not isoform A inhibits huntingtin aggregation. Has a stimulatory effect on the ATPase activity of HSP70 in a dose-dependent and time-dependent manner and hence acts as a co-chaperone of HSP70. Also reduces cellular toxicity and caspase-3 activity; DNAJ heat shock proteins
Synonyms: DNAJB6, DNAJB6p, hDNAJB6, C9J2C4, C9J2P2 ...