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  4. Recombinant Human Glucosidase 2 subunit beta (PRKCSH), partial

Recombinant Human Glucosidase 2 subunit beta (PRKCSH), partial

  • 货号：
    CSB-RP029254h
  • 规格：
    ￥1344
  • 图片：
    • (Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
  • 其他：

产品详情

  • 纯度：
    Greater than 90% as determined by SDS-PAGE.
  • 基因名：
  • Uniprot No.：
  • 别名：
    80K-H protein; AGE-binding receptor 2; AGE-R2; G19P1; GLU2B_HUMAN; Glucosidase 2 subunit beta; Glucosidase II beta subunit; Glucosidase II subunit beta; Hepatocystin; PCLD; PKCSH; PLD1; PRKCSH; Protein kinase C substrate 60.1 kDa protein heavy chain; Protein kinase C substrate 80 Kda protein; Protein kinase C substrate 80K-H; Protein kinase C substrate; 80 Kda protein
  • 种属：
    Homo sapiens (Human)
  • 蛋白长度：
    Partial
  • 来源：
    E.coli
  • 分子量：
    59.3kDa
  • 表达区域：
    15-302aa
  • 氨基酸序列
    VEVKRPRGVSLTNHHFYDESKPFTCLDGSATIPFDQVNDDYCDCKDGSDEPGTAACPNGSFHCTNTGYKPLYIPSNRVNDGVCDCCDGTDEYNSGVICENTCKEKGRKERESLQQMAEVTREGFRLKKILIEDWKKAREEKQKKLIELQAGKKSLEDQVEMLRTVKEEAEKPEREAKEQHQKLWEEQLAAAKAQQEQELAADAFKELDDDMDGTVSVTELQTHPELDTDGDGALSEAEAQALLSGDTQTDATSFYDRVWAAIRDKYRSEALPTDLPAPSAPDLTEPKE
    Note: The complete sequence including tag sequence, target protein sequence and linker sequence could be provided upon request.
  • 蛋白标签：
    N-terminal GST-tagged
  • 产品提供形式：
    Liquid or 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.
  • 缓冲液：
    Tris-based buffer,50% glycerol
  • 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：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. 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 & COA：
    Please contact us to get it.

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