Price range: $268.00 through $328.00
Background:
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],
| Target | Glucosidase IIβ |
| Reactivity | Human, Mouse |
| Applications | WB, IF, ELISA |
| MW(Calculated) | |
| MW(Observed) | 59kD |
| Host Species | Rabbit |
| Isotype | IgG |
| Conjugate/Modification | Unmodified |
| Modification | |
| Recommended Dilution Ratio | WB 1:500-1:2000; IF 1:200-1:1000; ELISA 1:10000; Not yet tested in other applications. |
| Formulation | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Source | |
| Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen. |
| Purity | |
| Storage | -15°C to -25°C/1 year(Do not lower than -25°C) |
| Concentration | 1 mg/ml |
| Clonality | Polyclonal |
| Clone Number | |
| Immunogen | The antiserum was produced against synthesized peptide derived from human GLU2B. AA range:81-130 |
| Sequence | |
| Specificity | Glucosidase IIβ Polyclonal Antibody detects endogenous levels of Glucosidase IIβ protein. |
| Gene Name | PRKCSH |
| Protein Name | Glucosidase 2 subunit beta |
| Other Name | PRKCSH; / G19P1; / Glucosidase 2 subunit beta; / 80K-H protein; / Glucosidase II subunit beta; / Protein kinase C substrate 60.1 kDa protein heavy chain; / PKCSH |
| Species | Human |
| Gene ID-1 | 5589 |
| Uniprot | P14314, |
| Species.1 | Mouse |
| Gene ID-2 | 19089 |
| Uniprot.1 | O08795 |
| Species.2 | |
| Gene ID-3 | |
| Uniprot.2 | |
| Organism-4 | |
| Gene ID-4 | |
| SwissProt-4 | |
| Background | 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], |
| Cellular Localization | Endoplasmic reticulum . |
| Tissue Expression | Lung,Lymphocyte,Platelet, |
| Signaling_pathway | Genetic Information Processing >> Folding, sorting and degradation >> Protein processing in endoplasmic reticulum |
| Research Areas | >>Protein processing in endoplasmic reticulum |
| Function | Disease:Defects in PRKCSH are a cause of polycystic liver disease (PCLD) [MIM:174050]. 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) [MIM:173900], which in a considerable but uncertain proportion of cases is associated with hepatic cysts.,Function:Regulatory subunit of glucosidase II.,pathway:Glycan metabolism; N-glycan metabolism.,similarity:Contains 1 PRKCSH domain.,similarity:Contains 2 EF-hand domains.,subunit:Heterodimer of a catalytic alpha subunit (GANAB) and a beta subunit (PRKCSH). Binds glycosylated PTPRC., |
| RRID |
