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).
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
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.
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
Target: PRKCSH
Specificity: Human
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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
- IGImmunochromatography
- 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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