PRKCSH

This gene encodes the beta-subunit of glucosidase II, an N-linked glycan-processing enzyme in the endoplasmic reticulum. The encoded protein is an acidic phosphoprotein known to be a substrate for protein kinase C. Mutations in this gene have been associated with the autosomal dominant polycystic liver disease. Alternative splicing results in multiple transcript variants.
Full Name
protein kinase C substrate 80K-H
Function
Regulatory subunit of glucosidase II that cleaves sequentially the 2 innermost alpha-1,3-linked glucose residues from the Glc2Man9GlcNAc2 oligosaccharide precursor of immature glycoproteins (PubMed:10929008).
Required for efficient PKD1/Polycystin-1 biogenesis and trafficking to the plasma membrane of the primary cilia (By similarity).
Biological Process
Intracellular signal transduction2 PublicationsNAS:UniProtKB
Liver developmentManual Assertion Based On ExperimentIBA:GO_Central
N-glycan processingManual Assertion Based On ExperimentIDA:UniProtKB
Cellular Location
Endoplasmic reticulum
Involvement in disease
Polycystic liver disease 1 with or without kidney cysts (PCLD1):
An autosomal dominant hepatobiliary disease characterized by overgrowth of biliary epithelium and supportive connective tissue, resulting in multiple liver cysts. A subset of patients may develop kidney cysts that usually do not result in clinically significant renal disease.
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Anti-PRKCSH antibodies

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Target: PRKCSH
Host: Mouse
Antibody Isotype: IgG2a, κ
Specificity: Human
Clone: 3H7
Application*: E
Target: PRKCSH
Host: Rabbit
Antibody Isotype: IgG
Specificity: Human
Clone: CBLG1-3097
Application*: WB
Target: PRKCSH
Host: Mouse
Antibody Isotype: IgG
Specificity: Mouse, Rat, Human
Clone: CBLG1-3241
Application*: WB
Target: PRKCSH
Host: Rabbit
Antibody Isotype: IgG
Specificity: Human, Mouse
Clone: EG1334
Application*: WB: 1:500~1:1000 IF: 1:100~1:500 ELISA: 1:10000
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For Research Use Only. Not For Clinical Use.
(P): Predicted
* Abbreviations
  • AActivation
  • AGAgonist
  • APApoptosis
  • BBlocking
  • BABioassay
  • BIBioimaging
  • CImmunohistochemistry-Frozen Sections
  • CIChromatin Immunoprecipitation
  • CTCytotoxicity
  • CSCostimulation
  • DDepletion
  • DBDot Blot
  • EELISA
  • ECELISA(Cap)
  • EDELISA(Det)
  • ESELISpot
  • EMElectron Microscopy
  • FFlow Cytometry
  • FNFunction Assay
  • GSGel Supershift
  • IInhibition
  • IAEnzyme Immunoassay
  • ICImmunocytochemistry
  • IDImmunodiffusion
  • IEImmunoelectrophoresis
  • IFImmunofluorescence
  • IHImmunohistochemistry
  • IMImmunomicroscopy
  • IOImmunoassay
  • IPImmunoprecipitation
  • ISIntracellular Staining for Flow Cytometry
  • LALuminex Assay
  • LFLateral Flow Immunoassay
  • MMicroarray
  • MCMass Cytometry/CyTOF
  • MDMeDIP
  • MSElectrophoretic Mobility Shift Assay
  • NNeutralization
  • PImmunohistologyp-Paraffin Sections
  • PAPeptide Array
  • PEPeptide ELISA
  • PLProximity Ligation Assay
  • RRadioimmunoassay
  • SStimulation
  • SESandwich ELISA
  • SHIn situ hybridization
  • TCTissue Culture
  • WBWestern Blot
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