DNAJB12


Description

The DNAJB12 (DnaJ heat shock protein family (Hsp40) member B12) is a protein-coding gene located on chromosome 10.

DNAJB12 functions as a co-chaperone alongside HSPA8/Hsc70, playing a vital role in promoting protein folding and trafficking, preventing aggregation of client proteins, and facilitating the degradation of unfolded proteins through the endoplasmic reticulum-associated degradation (ERAD) pathway. DNAJB12 influences HSPA8/Hsc70's ATPase and polypeptide-binding activities. Notably, DNAJB12 can also operate independently of HSPA8/Hsc70. In collaboration with DNAJB14, it acts as a chaperone, promoting the maturation of potassium channels KCND2 and KCNH2 by stabilizing nascent channel subunits and assembling them into tetramers. While the stabilization of these nascent channel proteins hinges on HSPA8/Hsc70, the oligomerization of channel subunits occurs independently of HSPA8/Hsc70. When overexpressed, DNAJB12 forms membranous structures within the nucleus, alongside DNAJB14 and HSPA8/Hsc70, known as DJANGOs. The precise function of these structures remains unclear.

DNAJB12 is also known as DJ10.

Associated Diseases



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