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DNAJB6 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. Defects in DNAJB6 are the cause of limb-girdle muscular dystrophy type 1E (LGMD1E). An autosomal dominant myopathy characterized by adult onset of proximal muscle weakness, beginning in the hip girdle region and later progressing to the shoulder girdle region. There is evidence that LGMD1E is caused by dysfunction of isoform B (PubMed:22366786). 3 isoforms of the human protein are produced by alternative splicing. Note: This description may include information from UniProtKB.
Protein type: Chaperone
Chromosomal Location of Human Ortholog: 7q36.3
Cellular Component: cytosol; membrane; nucleoplasm; nucleus; perinuclear region of cytoplasm; Z disc
Molecular Function: ATPase activator activity; chaperone binding; DNA binding; heat shock protein binding; protein binding; unfolded protein binding
Biological Process: actin cytoskeleton organization; extracellular matrix organization; intermediate filament organization; negative regulation of cysteine-type endopeptidase activity involved in apoptotic process; negative regulation of transcription, DNA-dependent; positive regulation of ATPase activity; protein folding; regulation of protein localization
Disease: Muscular Dystrophy, Limb-girdle, Type 1e
Reference #:  O75190 (UniProtKB)
Alt. Names/Synonyms: DJ4; DKFZp566D0824; DnaJ; DnaJ (Hsp40) homolog, subfamily B, member 6; DnaJ homolog subfamily B member 6; DnaJ-like 2 protein; DNAJB6; DNJB6; FLJ42837; Heat shock protein J2; HHDJ1; HSJ-2; HSJ2; MGC1152; MGC117297; MRJ; MSJ-1; MSJ1
Gene Symbols: DNAJB6
Molecular weight: 36,087 Da
Basal Isoelectric point: 9.17  Predict pI for various phosphorylation states
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Protein Structure Not Found.

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