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DNAJC5 Antibody

  • 货号：
    CSB-PA001802
  • 规格：
    ￥880
  • 图片：
    • Western Blot analysis of LOVO cells using CSP Polyclonal Antibody
  • 其他：

产品详情

  • Uniprot No.：
    Q9H3Z4
  • 基因名：
  • 别名：
    CLN 4 antibody; CLN4 antibody; CLN4B antibody; CSP antibody; cysteine string protein alpha antibody; Cysteine string protein antibody; DJC5 antibody; DnaJ (Hsp40) homolog subfamily C member 5 antibody; DnaJ homolog subfamily C member 5 antibody; DNAJC 5 antibody; Dnajc5 antibody; DNAJC5A antibody; DNJC5_HUMAN antibody; NCL antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse,Rat
  • 免疫原：
    Synthesized peptide derived from the C-terminal region of Human CSP.
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 抗体亚型：
    IgG
  • 纯化方式：
    The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
  • 产品提供形式：
    Liquid
  • 应用范围：
    WB, ELISA
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:500-1:2000
    ELISA 1:10000
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    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.

产品评价

靶点详情

  • 功能：
    Acts as a general chaperone in regulated exocytosis. Acts as a co-chaperone for the SNARE protein SNAP-25. Involved in the calcium-mediated control of a late stage of exocytosis. May have an important role in presynaptic function. May be involved in calcium-dependent neurotransmitter release at nerve endings.
  • 基因功能参考文献：
    1. Results indicate that by assisting local lysosome/proteasome processes, CSPalpha-mediated removal of toxic proteins via extracellular vesicles plays a central role in synaptic proteostasis. PMID: 28424476
    2. The importance of specific residues in the cysteine-string domain was investigated, revealing that a central core of palmitoylated cysteines is essential for aggregation of adult-onset neuronal ceroid lipofuscinosis CSPalpha L115R/L116 mutants. PMID: 28127059
    3. Phosphorylation of CSP triggers a major conformational switch that modulates its protein interactions. PMID: 27452402
    4. In fact, DnaJC5 overexpression induced tau release in cells, neurons, and brain tissue, but only when activity of the chaperone Hsc70 was intact and when tau was able to associate with this chaperone. PMID: 27261198
    5. This study demonstrated that Neuronal ceroid lipofuscinosis with DNAJC5/CSPalpha mutation has PPT1 pathology and exhibit aberrant protein palmitoylation. PMID: 26659577
    6. These results suggest that the degeneration seen in the patients with AD-ANCL reported here might be a consequence of both the early effects of CSPalpha mutations at the cellular soma. PMID: 26610600
    7. the presynaptic vesicle protein CSPalpha is a key player in synaptic degeneration and protection in Alzheimer's disease. PMID: 25631211
    8. Missense mutations in DNAJC5 does not play a major role in PD in the Chinese population. PMID: 24126164
    9. Results indicate of a p.L116del mutation in DNAJC5 from families with autosomal dominant Kufs disease. PMID: 22978711
    10. Palmitoylation-induced aggregation of mutant CSP-alpha proteins may underlie the development of adult-onset neuronal ceroid lipofuscinosis PMID: 22902780
    11. association of DNAJC5 mutations with autosomal dominant Kufs disease PMID: 22235333
    12. This is the first replication study of the identification of DNAJC5 as the disease-causing gene for autosomal dominant ANCL. The identification of the novel gene in ANCL will allow us to gain a better understanding of the pathological mechanism of ANCLs PMID: 22073189
    13. A neuroprotective role for CSPalpha in humans is confirmed. PMID: 21820099
    14. First evidence that CSP and HSP70, and their interactions with MARCKS, are involved in mucin secretion from airway epithelium. PMID: 18314541
    15. Csp not only regulates the exit of CFTR from the ER, but this action is accompanied by Hsc70/Hsp70 and CHIP-mediated CFTR degradation. PMID: 19098309
    16. Csp has a role in regulated CFTR trafficking at the plasma membrane. [CYSTEINE STRING PROTEIN] PMID: 12039948
    17. Cysteine string protein inhibits N-type calcium channels, but is blocked by mutant huntingtin PMID: 14570907
    18. CSP modulates G protein function by preferentially targeting the inactive GDP-bound form of G alpha(s) and promoting GDP/GTP exchange; the guanine nucleotide exchange activity of full-length CSP is regulated by Hsc70-SGT PMID: 15972823
    19. Cysteine string protein monitors late steps in cystic fibrosis transmembrane conductance regulator biogenesis PMID: 16469739
    20. palmitoylation of CSP is enhanced specifically by co-expression of the Golgi-localized palmitoyl transferases DHHC3, DHHC7, DHHC15, or DHHC17 PMID: 18596047
    21. Has a role in exocytosis of large dense core vesicles. PMID: 10194413

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  • 相关疾病：
    Ceroid lipofuscinosis, neuronal, 4B (CLN4B)
  • 亚细胞定位：
    Cytoplasm, cytosol. Membrane; Lipid-anchor. Cytoplasmic vesicle, secretory vesicle, chromaffin granule membrane. Melanosome. Cell membrane.
  • 组织特异性：
    Expressed in pancreas, kidney, skeletal muscle, liver, lung, placenta, brain and heart.
  • 数据库链接：

    HGNC: 16235

    OMIM: 162350

    KEGG: hsa:80331

    STRING: 9606.ENSP00000354111

    UniGene: Hs.164419