Glucosidase 2 subunit beta, Polyclonal Antibody

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

Polyclonal

IgG

Rabbit

Caprylic Acid Ammonium Sulfate Precipitation

Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C or -80 degree C. Avoid repeated freeze.

Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.

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.

Regulatory subunit of glucosidase II.

ELISA (EIA)

59,178 Da[Similar Products]

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

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

Disease: Polycystic Liver Disease

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