Recombinant Human Glucosidase 2 subunit beta(PRKCSH),partial

Code CSB-RP029254h
Size $1812Purchase it in Cusabio online store
(only available for customers from the US)
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  • (Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
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Product Details

Purity Greater than 90% as determined by SDS-PAGE.
Target Names PRKCSH
Uniprot No. P14314
Research Area Signal Transduction
Alternative Names 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
Species Homo sapiens (Human)
Source E.coli
Expression Region 15-302aa
Target Protein Sequence VEVKRPRGVSLTNHHFYDESKPFTCLDGSATIPFDQVNDDYCDCKDGSDEPGTAACPNGSFHCTNTGYKPLYIPSNRVNDGVCDCCDGTDEYNSGVICENTCKEKGRKERESLQQMAEVTREGFRLKKILIEDWKKAREEKQKKLIELQAGKKSLEDQVEMLRTVKEEAEKPEREAKEQHQKLWEEQLAAAKAQQEQELAADAFKELDDDMDGTVSVTELQTHPELDTDGDGALSEAEAQALLSGDTQTDATSFYDRVWAAIRDKYRSEALPTDLPAPSAPDLTEPKE
Note: The complete sequence including tag sequence, target protein sequence and linker sequence could be provided upon request.
Mol. Weight 59.3kDa
Protein Length Partial
Tag Info N-terminal GST-tagged
Form 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.
Buffer If the delivery form is liquid, the default storage buffer is Tris/PBS-based buffer, 5%-50% glycerol.
Note: If you have any special requirement for the glycerol content, please remark when you place the order.
If the delivery form is lyophilized powder, the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, pH 8.0.
Reconstitution 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°C/-80°C. Our default final concentration of glycerol is 50%. Customers could use it as reference.
Troubleshooting
and FAQs
Protein FAQs
Storage Condition Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
Shelf Life 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.
Lead Time Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Notes 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.

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Target Background

Function
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.
Gene References into Functions
  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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Involvement in disease Polycystic liver disease 1 (PCLD1)
Subcellular Location Endoplasmic reticulum.
Database Links

HGNC: 9411

OMIM: 174050

KEGG: hsa:5589

STRING: 9606.ENSP00000252455

UniGene: Hs.610830

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