PRKCSH Antibody

Code CSB-PA018709GA01HU
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Product Details

Uniprot No. P14314
Target Names PRKCSH
Alternative Names 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
Raised in Rabbit
Species Reactivity Human,Mouse,Rat
Immunogen Human PRKCSH
Immunogen Species Homo sapiens (Human)
Isotype IgG
Purification Method Antigen Affinity Purified
Concentration It differs from different batches. Please contact us to confirm it.
Buffer PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
Tested Applications ELISA,WB,IHC
Protocols ELISA Protocol
Western Blotting(WB) Protocol
Immunohistochemistry (IHC) Protocol
Troubleshooting and FAQs Antibody FAQs
Storage Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time 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.

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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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