DNAJB6 Antibody, FITC conjugated

說明書

中文名稱：

DNAJB6兔多克隆抗體, FITC偶聯
貨號：

CSB-PA03239C0Rb
規格：

￥880
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) DNAJB6 Polyclonal antibody
Uniprot No.：

O75190
基因名：

DNAJB6
別名：

DJ4 antibody; DnaJ antibody; DnaJ (Hsp40) homolog subfamily B member 6 antibody; DNAJ B6 antibody; DnaJ homolog subfamily B member 6 antibody; DnaJ like 2 protein antibody; DNAJB6 antibody; DNJB6_HUMAN antibody; Heat shock protein J2 antibody; HHDJ1 antibody; HSJ-2 antibody; HSJ2 antibody; LGMD1E antibody; MRJ antibody; MSJ-1 antibody; MSJ1 antibody
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human DnaJ homolog subfamily B member 6 protein (208-326AA)
免疫原種屬：

Homo sapiens (Human)
標記方式：

FITC
克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

>95%, Protein G purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
產品提供形式：

Liquid
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期：

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

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.
基因功能參考文獻：
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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相關疾?。?/div>
Limb-girdle muscular dystrophy 1E (LGMD1E)
亞細胞定位：

Cytoplasm, perinuclear region. Nucleus. Cytoplasm, myofibril, sarcomere, Z line.
組織特異性：

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
數據庫鏈接：

HGNC: 14888

OMIM: 603511

KEGG: hsa:10049

STRING: 9606.ENSP00000262177

UniGene: Hs.188591

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