ID:DNJB6_HUMAN DESCRIPTION: RecName: Full=DnaJ homolog subfamily B member 6; AltName: Full=HHDJ1; AltName: Full=Heat shock protein J2; Short=HSJ-2; AltName: Full=MRJ; AltName: Full=MSJ-1; 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. SUBUNIT: Homooligomer. Interacts with BAG3, HSPB8 and STUB1. Interacts with ALKBH1 (By similarity). Interacts with HSP70, KRT18 and PTTG. Isoform B interacts with histone deacetylases HDAC4, HDAC6, and SIRT2, HDAC activity is required for antiaggregation. INTERACTION: Q9HCU9:BRMS1; NbExp=2; IntAct=EBI-1053164, EBI-714781; O75923:DYSF; NbExp=2; IntAct=EBI-1053164, EBI-2799016; P05783:KRT18; NbExp=6; IntAct=EBI-1053164, EBI-297888; Q13326:SGCG; NbExp=2; IntAct=EBI-1053164, EBI-5357343; SUBCELLULAR LOCATION: Cytoplasm, perinuclear region. Nucleus. Cytoplasm, myofibril, sarcomere, Z line. 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 of differentiation pathway as well as in spermatids. In brain, expressed at a higher level in hippocampus and thalamus and a lower level in amygdala, substantia nigra, corpus callosum and caudate nucleus. DOMAIN: The antiaggregation activity of isoform B resides in the serine-rich region and the C-terminus. DISEASE: Defects in DNAJB6 are the cause of limb-girdle muscular dystrophy type 1E (LGMD1E) [MIM:603511]. 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. Note=There is evidence that LGMD1E is caused by dysfunction of isoform B (PubMed:22366786). SIMILARITY: Contains 1 J domain. SEQUENCE CAUTION: Sequence=AAD16010.1; Type=Frameshift; Positions=197; Sequence=BAD93096.1; Type=Erroneous initiation;
The RNAfold program from the Vienna RNA Package is used to perform the secondary structure predictions and folding calculations. The estimated folding energy is in kcal/mol. The more negative the energy, the more secondary structure the RNA is likely to have.
ModBase Predicted Comparative 3D Structure on O75190
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Orthologous Genes in Other Species
Orthologies between human, mouse, and rat are computed by taking the best BLASTP hit, and filtering out non-syntenic hits. For more distant species reciprocal-best BLASTP hits are used. Note that the absence of an ortholog in the table below may reflect incomplete annotations in the other species rather than a true absence of the orthologous gene.
Biological Process: GO:0006457 protein folding GO:0032781 positive regulation of ATPase activity GO:0032880 regulation of protein localization GO:0043154 negative regulation of cysteine-type endopeptidase activity involved in apoptotic process GO:0045109 intermediate filament organization GO:0090084 negative regulation of inclusion body assembly GO:1900034 regulation of cellular response to heat
AK301152 - Homo sapiens cDNA FLJ52214 complete cds, highly similar to DnaJ homolog subfamily B member 6. AK223601 - Homo sapiens mRNA for DnaJ (Hsp40) homolog, subfamily B, member 6 isoform a variant, clone: FCC131G10. BC110289 - Homo sapiens cDNA clone IMAGE:5185967, containing frame-shift errors. AL136707 - Homo sapiens mRNA; cDNA DKFZp566D0824 (from clone DKFZp566D0824). LF383954 - JP 2014500723-A/191457: Polycomb-Associated Non-Coding RNAs. AK297796 - Homo sapiens cDNA FLJ51000 complete cds, highly similar to DnaJ homolog subfamily B member 6. LP895577 - Sequence 441 from Patent EP3253886. BC002446 - Homo sapiens DnaJ (Hsp40) homolog, subfamily B, member 6, mRNA (cDNA clone MGC:1152 IMAGE:3346070), complete cds. AB209859 - Homo sapiens mRNA for DnaJ (Hsp40) homolog, subfamily B, member 6 isoform a variant protein. AB015799 - Homo sapiens HSJ2 mRNA for DnaJ homolog, complete cds. AF060703 - Homo sapiens DNAj homolog mRNA, complete cds. AB015798 - Homo sapiens HSJ2 mRNA for DnaJ homolog, complete cds. BC000177 - Homo sapiens DnaJ (Hsp40) homolog, subfamily B, member 6, mRNA (cDNA clone MGC:5201 IMAGE:2900421), complete cds. AK297497 - Homo sapiens cDNA FLJ60022 complete cds, highly similar to DnaJ homolog subfamily B member 6. EU075177 - Homo sapiens DnaJ mRNA, complete cds. AK291953 - Homo sapiens cDNA FLJ77164 complete cds, highly similar to Homo sapiens DnaJ (Hsp40) homolog, subfamily B, member 6 (DNAJB6), transcript variant 2, mRNA. AK293025 - Homo sapiens cDNA FLJ76979 complete cds, highly similar to Homo sapiens DnaJ (Hsp40) homolog, subfamily B, member 6 (DNAJB6), transcript variant 1, mRNA. AF075601 - Homo sapiens heat shock J2 protein (HSJ2) mRNA, complete cds. AF080569 - Homo sapiens DnaJ-like 2 protein (HSJ2) mRNA, complete cds. AK297989 - Homo sapiens cDNA FLJ51007 complete cds, highly similar to DnaJ homolog subfamily B member 6. AB014888 - Homo sapiens mRNA for MRJ, complete cds. AM392741 - Synthetic construct Homo sapiens clone IMAGE:100001796 for hypothetical protein (DNAJB6 gene). AM393348 - Synthetic construct Homo sapiens clone IMAGE:100001802 for hypothetical protein (DNAJB6 gene). AB385552 - Synthetic construct DNA, clone: pF1KB5520, Homo sapiens DNAJB6 gene for DnaJ homolog, subfamily B, member 6, complete cds, without stop codon, in Flexi system. AM392548 - Synthetic construct Homo sapiens clone IMAGE:100002118 for hypothetical protein (DNAJB6 gene). KJ892903 - Synthetic construct Homo sapiens clone ccsbBroadEn_02297 DNAJB6 gene, encodes complete protein. CR533498 - Homo sapiens full open reading frame cDNA clone RZPDo834F0317D for gene DNAJB6, DnaJ (Hsp40) homolog, subfamily B, member 6; complete cds, incl. stopcodon. MA619531 - JP 2018138019-A/191457: Polycomb-Associated Non-Coding RNAs. JD364806 - Sequence 345830 from Patent EP1572962. JD396448 - Sequence 377472 from Patent EP1572962. JD518905 - Sequence 499929 from Patent EP1572962. AL832124 - Homo sapiens mRNA; cDNA DKFZp686C0910 (from clone DKFZp686C0910). BC043270 - Homo sapiens DnaJ (Hsp40) homolog, subfamily B, member 6, mRNA (cDNA clone IMAGE:5297025), complete cds. AK124827 - Homo sapiens cDNA FLJ42837 fis, clone BRCAN2021024, weakly similar to DnaJ homolog subfamily B member 6. AK055429 - Homo sapiens cDNA FLJ30867 fis, clone FEBRA2004191. AK093837 - Homo sapiens cDNA FLJ36518 fis, clone TRACH2002054. JD106516 - Sequence 87540 from Patent EP1572962. JD354418 - Sequence 335442 from Patent EP1572962. JD460942 - Sequence 441966 from Patent EP1572962. JD560616 - Sequence 541640 from Patent EP1572962. JD458661 - Sequence 439685 from Patent EP1572962. JD259593 - Sequence 240617 from Patent EP1572962. JD520533 - Sequence 501557 from Patent EP1572962. JD433206 - Sequence 414230 from Patent EP1572962. JD539463 - Sequence 520487 from Patent EP1572962. JD235280 - Sequence 216304 from Patent EP1572962. JD486639 - Sequence 467663 from Patent EP1572962. JD046544 - Sequence 27568 from Patent EP1572962. JD224898 - Sequence 205922 from Patent EP1572962. JD314945 - Sequence 295969 from Patent EP1572962. JD326778 - Sequence 307802 from Patent EP1572962. JD265603 - Sequence 246627 from Patent EP1572962. JD227147 - Sequence 208171 from Patent EP1572962. BC018175 - Homo sapiens, clone IMAGE:3865615, mRNA. JD560835 - Sequence 541859 from Patent EP1572962. JD089877 - Sequence 70901 from Patent EP1572962. JD264325 - Sequence 245349 from Patent EP1572962. JD039520 - Sequence 20544 from Patent EP1572962. JD298694 - Sequence 279718 from Patent EP1572962. JD503661 - Sequence 484685 from Patent EP1572962. JD111249 - Sequence 92273 from Patent EP1572962.
Biochemical and Signaling Pathways
BioCarta from NCI Cancer Genome Anatomy Project h_prionPathway - Prion Pathway
Reactome (by CSHL, EBI, and GO)
Protein O75190 (Reactome details) participates in the following event(s):
R-HSA-5251959 HSP40s activate intrinsic ATPase activity of HSP70s in the cytosol R-HSA-5251955 HSP40s activate intrinsic ATPase activity of HSP70s in the nucleoplasm R-HSA-3371453 Regulation of HSF1-mediated heat shock response R-HSA-3371556 Cellular response to heat stress R-HSA-2262752 Cellular responses to stress R-HSA-8953897 Cellular responses to external stimuli
US Server
Sequence and Links to Tools and Databases 
Common Gene Haplotype Alleles 
