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  4. Recombinant Human DnaJ homolog subfamily B member 6 (DNAJB6)

Recombinant Human DnaJ homolog subfamily B member 6 (DNAJB6)

  • 货号：
    CSB-YP007012HU
  • 规格：
  • 来源：
    Yeast
  • 其他：
  • 货号：
    CSB-EP007012HU
  • 规格：
  • 来源：
    E.coli
  • 其他：
  • 货号：
    CSB-EP007012HU-B
  • 规格：
  • 来源：
    E.coli
  • 共轭：
    Avi-tag Biotinylated

    E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.

  • 其他：
  • 货号：
    CSB-BP007012HU
  • 规格：
  • 来源：
    Baculovirus
  • 其他：
  • 货号：
    CSB-MP007012HU
  • 规格：
  • 来源：
    Mammalian cell
  • 其他：

产品详情

  • 纯度：
    >85% (SDS-PAGE)
  • 基因名：
    DNAJB6
  • Uniprot No.：
  • 别名：
    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
  • 种属：
    Homo sapiens (Human)
  • 蛋白长度：
    Full Length of Mature Protein
  • 表达区域：
    2-326
  • 氨基酸序列
    VDYYEVLGV QRHASPEDIK KAYRKLALKW HPDKNPENKE EAERKFKQVA EAYEVLSDAK KRDIYDKYGK EGLNGGGGGG SHFDSPFEFG FTFRNPDDVF REFFGGRDPF SFDFFEDPFE DFFGNRRGPR GSRSRGTGSF FSAFSGFPSF GSGFSSFDTG FTSFGSLGHG GLTSFSSTSF GGSGMGNFKS ISTSTKMVNG RKITTKRIVE NGQERVEVEE DGQLKSLTIN GVADDDALAE ERMRRGQNAL PAQPAGLRPP KPPRPASLLR HAPHCLSEEE GEQDRPRAPG PWDPLASAAG LKEGGKRKKQ KQREESKKKK STKGNH
  • 蛋白标签：
    Tag type will be determined during the manufacturing process.
    The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
  • 产品提供形式：
    Lyophilized powder
    Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
  • 复溶：
    We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
  • 储存条件：
    Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
  • 保质期：
    The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
    Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
  • 货期：
    Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
    Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
  • 注意事项：
    Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
  • Datasheet ：
    Please contact us to get it.

产品评价

靶点详情

  • 功能：
    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.
  • 基因功能参考文献：
    1. 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
    2. This study expands the molecular spectrum of DNAJB6 mutations and also emphasizes the pathogenic role of DNAJB6 dysfunction in distal-onset myopathy. PMID: 28233300
    3. 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
    4. mRNA levels of HSP family members (HSP70B', HSP72, HSP40/DNAJ, and HSP20/CRYAB) are upregulated by the intracellular MMP3 overload. PMID: 27206651
    5. the cytoprotective effects of DNAJB6(S) may be mediated, at least in part, by the mitochondrial pathway of apoptosis. PMID: 28280525
    6. 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
    7. LGMD1D mutations in DNAJB6 disrupt its sarcoplasmic function suggesting a role for DNAJB6b in Z-disc organization and stress granule kinetics. PMID: 26362252
    8. 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
    9. 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
    10. 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
    11. This study showed that Complete loss of the DNAJB6 G/F domain and novel missense mutations cause distal-onset DNAJB6 myopathy. PMID: 26205529
    12. 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
    13. There was a positive correlation between DNAJB6 and IQGAP1 expression. PMID: 25044025
    14. Geneticanalysis indicated a heterozygous missense mutation of c.279C>G PMID: 25306414
    15. 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
    16. DNAJB6 G/F domain mutants disrupt the processing of nuclear TDP-43 stress granules in mammalian cells. PMID: 24920671
    17. DNAJB6 interacts with growing amyloid-beta 42 (Abeta42) aggregates, which leads to sub-stoichiometric inhibition of amyloid formation PMID: 25217638
    18. 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
    19. DNAJB6 is a peptide-binding chaperone that can interact with polyQ peptides that are incompletely degraded by and released from the proteasome. PMID: 23904097
    20. have previously reported clinical, genetic and molecular pathomechanistic findings in DNAJB6 mutated LGMD1D PMID: 23865856
    21. 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
    22. We identified DNAJ/HSP40B6 as a potential negative regulator of HIV-1 replication in our genetic screens. PMID: 24047968
    23. 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
    24. The mechanism of DNAJB6 and DNAJB8 is suppression of polyQ protein aggregation by directly binding the polyQ tract. PMID: 23612975
    25. 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
    26. 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
    27. A novel regulatory mechanism for DNAJB6-mediated DKK1 transcriptional up-regulation might influence epithelial-mesenchymal transition. PMID: 22455953
    28. MRJ (short form) shows nuclear localization signal independent nuclear localization in response to heat shock and hypoxia. PMID: 22504047
    29. 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
    30. Mutations within the Pro96Arg domain of DNAJB6 are a novel cause of dominantly-inherited myopathy. PMID: 22334415
    31. the interaction between urokinase receptor and heat shock protein MRJ enhances cell adhesion PMID: 20372789
    32. DNAJB6 induces degradation of beta-catenin and causes partial reversal of mesenchymal phenotype PMID: 20522561
    33. DNAJB6b and DNAJB8 are superior suppressors of aggregation and toxicity of disease-associated polyglutamine proteins. PMID: 20159555
    34. MRJ has a relevant functional role in neurons. PMID: 11896048
    35. Role of Hsp40 co-chaperone Hdj-1 in CFTR turnover with HSP70 PMID: 12069690
    36. NFATc3 is negatively regulated by class II histone deacetylases through the DnaJ (heat shock protein-40) superfamily member Mrj PMID: 16260608
    37. msj-1 gene might be conserved among vertebrates and might exert fundamental functions in reproduction. PMID: 18184612
    38. 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
    39. 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
    40. 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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  • 相关疾病：
    Limb-girdle muscular dystrophy 1E (LGMD1E)
  • 亚细胞定位：
    Cytoplasm, perinuclear region. Nucleus. Cytoplasm, myofibril, sarcomere, Z line.
  • 组织特异性：
    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
  • 数据库链接：

    HGNC: 14888

    OMIM: 603511

    KEGG: hsa:10049

    STRING: 9606.ENSP00000262177

    UniGene: Hs.188591