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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.
Gene References into Functions
DNAJB6 is a central and versatile player in the protein aggregation and degradation system.DNAJB6 protein keeps Parkin C289G mutant protein in a soluble, degradation-competent form. PMID: 27713507
This study expands the molecular spectrum of DNAJB6 mutations and also emphasizes the pathogenic role of DNAJB6 dysfunction in distal-onset myopathy. PMID: 28233300
Expression of the heat shock protein DNAJB6/MRJ was elevated in neutrophils and lymphocytes of patients with atopic dermatitis compared with healthy donors. The highest level of the DNAJB6/MRJ protein was found to be in neutrophils at acute phase of severe atopic dermatitis and gradually decline as continue to the disease. PMID: 29244458
mRNA levels of HSP family members (HSP70B', HSP72, HSP40/DNAJ, and HSP20/CRYAB) are upregulated by the intracellular MMP3 overload. PMID: 27206651
the cytoprotective effects of DNAJB6(S) may be mediated, at least in part, by the mitochondrial pathway of apoptosis. PMID: 28280525
The results indicate both genetic and physical interactions between disease-linked RNA-binding proteinss and DNAJB6/mrj, suggesting etiologic overlap between the pathogenesis of adult-onset inherited myopathies initiated by mutations in hnRNPA2B1 and DNAJB6. PMID: 26744327
LGMD1D mutations in DNAJB6 disrupt its sarcoplasmic function suggesting a role for DNAJB6b in Z-disc organization and stress granule kinetics. PMID: 26362252
DNAJB6 mutations p.F91I and p.F91L show a significant reduction of the anti-aggregation function compared to the wild-type and p.F93L mutation PMID: 26338452
Functional study using zebrafish embryos demonstrated that p.Phe91Leu elicits more severe muscle defects than the reported p.Phe93Leu and p.Pro96Arg mutations PMID: 26371419
DnaJB6-protected yeast cells from polyglutamine toxicity and cured yeast of both [URE3] prions and weak variants of [PSI(+)] prions but not strong [PSI(+)] prions PMID: 26702057
This study showed that Complete loss of the DNAJB6 G/F domain and novel missense mutations cause distal-onset DNAJB6 myopathy. PMID: 26205529
DNAJB6a reduces AKT signaling, and DNAJB6 expression in cancer cells reduces their proliferation and growth of xenograft esophageal squamous cell tumors in mice. PMID: 26302489
There was a positive correlation between DNAJB6 and IQGAP1 expression. PMID: 25044025
Geneticanalysis indicated a heterozygous missense mutation of c.279C>G PMID: 25306414
Findings suggest a novel function of HSP70/MRJ/uPAR complex in cell adhesion, invasion and migration, and may provide more understanding in the mechanisms of uPAR-mediated cancer metastasis. PMID: 25175595
DNAJB6 G/F domain mutants disrupt the processing of nuclear TDP-43 stress granules in mammalian cells. PMID: 24920671
DNAJB6 interacts with growing amyloid-beta 42 (Abeta42) aggregates, which leads to sub-stoichiometric inhibition of amyloid formation PMID: 25217638
using exome sequencing, study identified a mutation in DNAJB6 in a family with limb-girdle muscular dystrophy type 1D; work further confirms the causative role of DNAJB6 mutations in limb-girdle muscular dystrophy type 1D PMID: 24594375
DNAJB6 is a peptide-binding chaperone that can interact with polyQ peptides that are incompletely degraded by and released from the proteasome. PMID: 23904097
have previously reported clinical, genetic and molecular pathomechanistic findings in DNAJB6 mutated LGMD1D PMID: 23865856
A 56-year-old woman, like 3 other family members, becomes symptomatic in childhood with slowly progressive limb-girdle muscle weakness, normal serum creatine kinase (CK) values, and myopathic electromyographic findings. PMID: 24170373
We identified DNAJ/HSP40B6 as a potential negative regulator of HIV-1 replication in our genetic screens. PMID: 24047968
we detected cytoplasmic accumulations associated with chaperone-assisted selective autophagy together with intranuclear accumulations of DNAJB6 and HSPB8. This is the first report of Asian patients with limb-girdle muscular dystrophy type 1D PMID: 23394708
The mechanism of DNAJB6 and DNAJB8 is suppression of polyQ protein aggregation by directly binding the polyQ tract. PMID: 23612975
the relative expression levels of DNAJB6 isoforms may play a key role in regulating the cellular localization of UL70, leading to modulation of HCMV DNA synthesis and lytic infection. PMID: 23133382
miR-632 is a potentially important epigenetic regulator of DNAJB6, which contributes to the downregulation of DNAJB6 and plays a supportive role in malignant progression PMID: 22710984
A novel regulatory mechanism for DNAJB6-mediated DKK1 transcriptional up-regulation might influence epithelial-mesenchymal transition. PMID: 22455953
MRJ (short form) shows nuclear localization signal independent nuclear localization in response to heat shock and hypoxia. PMID: 22504047
studied 9 Limb-girdle muscular dystrophy type 1D affected families from Finland, the United States and Italy and identified 4 dominant missense mutations leading to p.Phe93Leu or p.Phe89Ile changes in the co-chaperone DNAJB6 PMID: 22366786
Mutations within the Pro96Arg domain of DNAJB6 are a novel cause of dominantly-inherited myopathy. PMID: 22334415
the interaction between urokinase receptor and heat shock protein MRJ enhances cell adhesion PMID: 20372789
DNAJB6 induces degradation of beta-catenin and causes partial reversal of mesenchymal phenotype PMID: 20522561
DNAJB6b and DNAJB8 are superior suppressors of aggregation and toxicity of disease-associated polyglutamine proteins. PMID: 20159555
MRJ has a relevant functional role in neurons. PMID: 11896048
Role of Hsp40 co-chaperone Hdj-1 in CFTR turnover with HSP70 PMID: 12069690
NFATc3 is negatively regulated by class II histone deacetylases through the DnaJ (heat shock protein-40) superfamily member Mrj PMID: 16260608
msj-1 gene might be conserved among vertebrates and might exert fundamental functions in reproduction. PMID: 18184612
Large isoform of MRJ(L), DNAJB6, is a nuclear protein that is lost in breast cancer, that regulates several key players in tumor formation and metastasis, and that is functionally able to retard tumor growth. PMID: 18328103
DnaJB6 is necessary for translocation of Slfn1 into the nucleus, where Slfn1 down-regulates cyclin D1, induces cell-cycle arrest and programmes a quiescent state of T-cells PMID: 18373498
we report the up-regulation of Mrj protein in M-phase of HeLa cells implicating its role in mitosis related activities. PMID: 19002655
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Involvement in disease
Limb-girdle muscular dystrophy 1E (LGMD1E)
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