1004923950ENSG00000105993ENSMUSG00000029131O75190O54946NM_005494NM_058246NM_001363676NM_001378835NP_005485NP_490647NP_001350605NP_001365764DnaJ homolog subfamily B member 6 is a protein that in humans is encoded by the DNAJB6 gene.
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.
[8] It has been also shown that the aggregation of Aβ42 (a process involved in e.g. Alzheimer's disease) is retarded by DNAJB6 in a concentration-dependent manner, extending to very low sub-stoichiometric molar ratios of chaperone to peptide.
[9] Dominant mutations in DNAJB6 have also been found to cause a late-onset muscle disease termed limb-girdle muscular dystrophy type D1 (LGMDD1), which is characterized by protein aggregation and vacuolar myopathology.