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PRKCSH Antibody

  • 货号：
    CSB-PA018709GA01HU
  • 规格：
    ￥3,900
  • 其他：

产品详情

  • Uniprot No.：
    P14314
  • 基因名：
  • 别名：
    80K-H protein antibody; AGE-binding receptor 2 antibody; AGE-R2 antibody; G19P1 antibody; GLU2B_HUMAN antibody; Glucosidase 2 subunit beta antibody; Glucosidase II beta subunit antibody; Glucosidase II subunit beta antibody; Hepatocystin antibody; PCLD antibody; PKCSH antibody; PLD1 antibody; PRKCSH antibody; Protein kinase C substrate 60.1 kDa protein heavy chain antibody; Protein kinase C substrate 80 Kda protein antibody; Protein kinase C substrate 80K-H antibody; Protein kinase C substrate; 80 Kda protein antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse,Rat
  • 免疫原：
    Human PRKCSH
  • 免疫原种属：
    Homo sapiens (Human)
  • 抗体亚型：
    IgG
  • 纯化方式：
    Antigen Affinity Purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA,WB,IHC
  • 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：
    Regulatory subunit of glucosidase II that cleaves sequentially the 2 innermost alpha-1,3-linked glucose residues from the Glc(2)Man(9)GlcNAc(2) oligosaccharide precursor of immature glycoproteins. Required for efficient PKD1/Polycystin-1 biogenesis and trafficking to the plasma membrane of the primary cilia.
  • 基因功能参考文献：
    1. Results suggest that loss of PRKCSH and SEC63 leads to general defects in ciliogenesis, while quenching of the Wnt signaling cascade is cholangiocyte-restricted. PMID: 28973524
    2. This study demonstrated that Large copy number variations on germline level are not present in patients with a clinical diagnosis of Severe Polycystic Liver Disease. PMID: 26365003
    3. Polycystic liver disease is recessive at the cellular level, and loss of functional PRKCSH is an important step in cystogenesis. PMID: 21856269
    4. The induction of autophagy by hepatocystin deficiency is mediated through mammalian target of rapamycin (mTOR). PMID: 21681021
    5. Results provide evidence that mutations at the coding PRKCSH GAG repeat are a target of MSI and are selectively associated with the MSI-H phenotype in gastric carcinomas. PMID: 21371016
    6. The common SNPs tested in DDOST, PRKCSH and LGALS3 do not seem to be associated with diabetic micro- or macrovascular complications or with type 1 diabetes in Finnish patients. PMID: 20490454
    7. identified a total of 26 novel mutations in PRKCSH (n = 14) and SEC63 (n = 12), including four splice site mutations, eight insertions/ deletions, six non-sense mutations, and eight missense mutations PMID: 20095989
    8. Our results suggest that PRKCSH gene is not a major genetic cause of PCLD and there may be at least another locus responsible for the disease in Taiwan. PMID: 19308730
    9. PRKCSH functions as a chaperone-like molecule, which prevents endoplasmic reticulum-associated degradation of TRPP2. PMID: 19801576
    10. Mutations in this protein cause isolated autosomal dominant polycystic liver disease. PMID: 12529853
    11. germline mutations in PRKCSH as the probable cause of autosomal dominant polycystic liver disease PMID: 12577059
    12. autosomal dominant polycystic liver disease is genetically heterogeneous PMID: 15057895
    13. role of hepatocystin in carbohydrate processing and quality control of newly synthesized glycoproteins in the endoplasmic reticulum PMID: 15188177
    14. results identify 80K-H as a new player involved in GLUT4 vesicle transport and identify a link between a kinase involved in the insulin signalling cascade, PKCzeta, and a known component of the GLUT4 vesicle trafficking pathway, munc18c PMID: 15707389
    15. the majority of cysts from PRKCSH mutation carriers did not express hepatocystin PMID: 18224332
    16. Hepatocystin is not secreted in liver cyst fluids of autosomal dominant polycystic liver disease patients, suggesting that mutant hepatocystin is either not produced or degraded intracellularly. PMID: 18419150
    17. 80K-H is a novel regulator of IP3R1 activity, and it may contribute to neuronal functions. PMID: 18990696
    18. These results indicate that insulin induces dynamic associations between PKCzeta, 80K-H, and munc18c and that 80K-H may act as a key signaling link between PKCzeta and munc18c in live cells. PMID: 19061073

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  • 相关疾病：
    Polycystic liver disease 1 (PCLD1)
  • 亚细胞定位：
    Endoplasmic reticulum.
  • 数据库链接：

    HGNC: 9411

    OMIM: 174050

    KEGG: hsa:5589

    STRING: 9606.ENSP00000252455

    UniGene: Hs.610830