DnaJ homolog subfamily B member 6

Definition:

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. 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. Also reduces cellular toxicity and caspase-3 activity.; [Isoform B]: Isoform B but not isoform A inhibits huntingtin aggregation.

References:

[1]. Jurre Hageman, et al. A DNAJB chaperone subfamily with HDAC-dependent activities suppresses toxic protein aggregation. Mol Cell. 2010 Feb 12;37(3):355-69. [Content Brief]

[2]. I Izawa, et al. Identification of Mrj, a DnaJ/Hsp40 family protein, as a keratin 8/18 filament regulatory protein. J Biol Chem. 2000 Nov 3;275(44):34521-7. [Content Brief]

[3]. P-C Tsai, et al. A novel DNAJB6 mutation causes dominantly inherited distal-onset myopathy and compromises DNAJB6 function. Clin Genet. 2017 Aug;92(2):150-157. [Content Brief]

[4]. Jaakko Sarparanta, et al. Mutations affecting the cytoplasmic functions of the co-chaperone DNAJB6 cause limb-girdle muscular dystrophy. Nat Genet. 2012 Feb 26;44(4):450-5, S1-2. [Content Brief]

[5]. Jen-Zen Chuang, et al. Characterization of a brain-enriched chaperone, MRJ, that inhibits Huntingtin aggregation and toxicity independently. J Biol Chem. 2002 May 31;277(22):19831-8. [Content Brief]

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