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.

Anti-PRKCSH antibodies

Rabbit Anti-PRKCSH Recombinant Antibody (CBLG1-3097) (CBMAB-G6476-LY)

Datasheet

SDS

Target: PRKCSH

Host: Rabbit

Antibody Isotype: IgG

Specificity: Human

Clone: CBLG1-3097

Application*: WB

Mouse Anti-PRKCSH Recombinant Antibody (CBLG1-3241) (CBMAB-G6474-LY)

Datasheet

SDS

Target: PRKCSH

Host: Mouse

Antibody Isotype: IgG

Specificity: Mouse, Rat, Human

Clone: CBLG1-3241

Application*: WB

Rabbit Anti-PRKCSH Recombinant Antibody (EG1334) (CBMAB-EN1612-LY)

Datasheet

SDS

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

Mouse Anti-PRKCSH Recombinant Antibody (3H7) (CBMAB-P2857-YC)

Datasheet

SDS

Target: PRKCSH

Host: Mouse

Antibody Isotype: IgG2a, κ

Specificity: Human

Clone: 3H7

Application*: E

PRKCSH Matched Antibody Pair (928) (APMAB-928LY)

Datasheet

SDS

Target: PRKCSH

Specificity: Human

For Research Use Only. Not For Clinical Use.

(P): Predicted

* Abbreviations

IFImmunofluorescence

IHImmunohistochemistry

IPImmunoprecipitation

WBWestern Blot

EELISA

MMicroarray

CIChromatin Immunoprecipitation

FFlow Cytometry

FNFunction Assay

IDImmunodiffusion

RRadioimmunoassay

TCTissue Culture

GSGel Supershift

NNeutralization

BBlocking

AActivation

IInhibition

DDepletion

ESELISpot

DBDot Blot

MCMass Cytometry/CyTOF

CTCytotoxicity

SStimulation

AGAgonist

APApoptosis

IMImmunomicroscopy

BABioassay

CSCostimulation

EMElectron Microscopy

IEImmunoelectrophoresis

PAPeptide Array

ICImmunocytochemistry

PEPeptide ELISA

MDMeDIP

SHIn situ hybridization

IAEnzyme Immunoassay

SEsandwich ELISA

PLProximity Ligation Assay

ECELISA(Cap)

EDELISA(Det)

BIBioimaging

IOImmunoassay

LFLateral Flow Immunoassay

LALuminex Assay

CImmunohistochemistry-Frozen Sections

PImmunohistologyp-Paraffin Sections

ISIntracellular Staining for Flow Cytometry

MSElectrophoretic Mobility Shift Assay

RIRNA Binding Protein Immunoprecipitation (RIP)