Rabbit anti-Human PRKCSH Polyclonal Antibody | anti-PRKCSH antibody

PRKCSH, NT (PRKCSH, G19P1, Glucosidase 2 subunit beta, 80K-H protein, Glucosidase II subunit beta, Protein kinase C substrate 60.1kD protein heavy chain) (AP)

Cat. Num. AAA6337369

• Gene Names: PRKCSH; PCLD; PLD1; G19P1; PKCSH; AGE-R2
• Reactivity: Human
• Applications: Western Blot, ELISA
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: PRKCSH; Polyclonal Antibody; NT (PRKCSH; G19P1; Glucosidase 2 subunit beta; 80K-H protein; Glucosidase II subunit beta; Protein kinase C substrate 60.1kD protein heavy chain) (AP); Protein kinase C substrate 60.1kD protein heavy chain; anti-PRKCSH antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Alkaline Phosphatase (AP).

• Applicable Applications for anti-PRKCSH antibody: Western Blot (WB), ELISA (EIA)
• Application Notes: WB: 1:100-500; ELISA: 1:1,000; Applications are based on unconjugated antibody.
• Immunogen: PRKCSH antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 5-35 amino acids from the N-terminal region of human PRKCSH.
• Conjugate: AP
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

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

Related Product Information for anti-PRKCSH antibody

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.

Product Categories/Family for anti-PRKCSH antibody

Antibodies; Protein Kinases

References

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).

NCBI and Uniprot Product Information

• NCBI GI #: 48255889
• NCBI GeneID: 5589
• NCBI Accession #: NP_002734.2
• NCBI GenBank Nucleotide #: NM_002743.3
• UniProt Accession #: P14314; Q96BU9; Q96D06; Q9P0W9; A8K318
• Molecular Weight: 59,178 Da
• NCBI Official Full Name: glucosidase 2 subunit beta isoform 1
• NCBI Official Synonym Full Names: protein kinase C substrate 80K-H
• NCBI Official Symbol: PRKCSH
• NCBI Official Synonym Symbols: PCLD; PLD1; G19P1; PKCSH; AGE-R2
• NCBI Protein Information: glucosidase 2 subunit beta; hepatocystin; AGE-binding receptor 2; glucosidase II subunit beta; protein kinase C substrate, 80 Kda protein; protein kinase C substrate 60.1 kDa protein heavy chain
• UniProt Protein Name: Glucosidase 2 subunit beta
• Protein Family: Glucosidase
• UniProt Gene Name: PRKCSH
• UniProt Synonym Gene Names: G19P1; PKCSH
• UniProt Entry Name: GLU2B_HUMAN

Uniprot Description

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