Recombinant Human DnaJ homolog subfamily B member 6 (DNAJB6)

Uniprot Id

O75190

Target Species

Human

Target Name

DNAJB6

Target Full Name

DnaJ homolog subfamily B member 6

Target Function

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.

Target Involvement

Limb-girdle muscular dystrophy 1E (LGMD1E)

Target Subcellular Location

Cytoplasm, perinuclear region. Nucleus. Cytoplasm, myofibril, sarcomere, Z line.

Target Tissue Specificity

Widely expressed. Highest levels in testis and brain, and lower levels in heart, spleen, intestine, ovary, placenta, lung, kidney, pancreas, thymus, prostate, skeletal muscle, liver and leukocytes. In testis, expressed in germ cells in the earlier stages

Target Synonyms

DJ4 ; DnaJ ; DnaJ (Hsp40) homolog subfamily B member 6; DNAJ B6; DnaJ homolog subfamily B member 6; DnaJ like 2 protein; DNAJB6; DNJB6_HUMAN; Heat shock protein J2; HHDJ1; HSJ-2; HSJ2 ; LGMD1E; MRJ; MSJ-1; MSJ1

Target Background

This gene encodes a member of the DNAJ protein family. DNAJ family members are characterized by a highly conserved amino acid stretch called the 'J-domain' and function as one of the two major classes of molecular chaperones involved in a wide range of cellular events, such as protein folding and oligomeric protein complex assembly. This family member may also play a role in polyglutamine aggregation in specific neurons. Alternative splicing of this gene results in multiple transcript variants; however, not all variants have been fully described.