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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.
Gene Name: | DnaJ (Hsp40) homolog, subfamily B, member 6 |
Synonyms: | DNAJB6, DnaJ-like 2 protein, HSJ2, LGMD1E, MRJ, MSJ-1, MSJ1, DJ4, DnaJ, Heat shock protein J2, HHDJ1, HSJ-2 |
Target Sequences: | NM_005494 NP_005485.1 O75190 |
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