PRKCSH, Polyclonal Antibody

Glucosidase 2 subunit beta; 80K-H protein; Glucosidase II subunit beta; Protein kinase C substrate 601 kDa protein heavy chain; PKCSH; PRKCSH; G19P1

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit Ig

Rabbit

This PRKCSH antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 6-35 amino acids from the N-terminal region of human PRKCSH.

Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Vial Concentration: 0.33 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-PRKCSH antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

This gene encodes the beta-subunit of glucosidase II, an
N-linked glycan-processing enzyme in the endoplasmic reticulum
(ER). This protein is an acidic phospho-protein known to be a
substrate for protein kinase C. Mutations in this gene have been
associated with the autosomal dominant polycystic liver disease
(PCLD). Alternatively spliced transcript variants encoding distinct
isoforms have been observed.

Signal Transduction

Western Blot (WB), ELISA (EIA)

WB~~1:1000

PRKCSH Antibody (N-term) western blot analysis in Hela cell line lysates (35ug/lane).This demonstrates the PRKCSH antibody detected the PRKCSH protein (arrow).

59425

protein kinase C substrate 80K-H

glucosidase 2 subunit beta

Glucosidase 2 subunit beta

G19P1; PKCSH  [Similar Products]

GLU2B_HUMAN

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. [provided by RefSeq, Jan 2014]

PRKCSH: Regulatory subunit of glucosidase II. Defects in PRKCSH are a cause of polycystic liver disease (PCLD). PCLD is an autosomal dominant disorder and is characterized by the presence of multiple liver cysts of biliary epithelial origin. PCLD is a distinct clinical and genetic entity that can occur independently from autosomal dominant polycystic kidney disease (ADPKD), which in a considerable but uncertain proportion of cases is associated with hepatic cysts. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Adaptor/scaffold

Chromosomal Location of Human Ortholog: 19p13.2

Cellular Component: endoplasmic reticulum; endoplasmic reticulum lumen; intracellular

Molecular Function: protein kinase C binding; phosphoprotein binding; calcium ion binding

Biological Process: cellular protein metabolic process; protein folding; innate immune response; protein amino acid N-linked glycosylation via asparagine; N-glycan processing; post-translational protein modification

Disease: Polycystic Liver Disease

Hoverfelt, A., et al. Diabetologia 53(9):1903-1907(2010)
Waanders, E., et al. Clin. Genet. 78(1):47-56(2010)
van Keimpema, L., et al. Liver Int. (2010) In press :
Yang, A.M., et al. Dig. Dis. Sci. 55(3):815-819(2010)
Gao, H., et al. Hum. Mol. Genet. 19(1):16-24(2010)

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