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Description
This gene encodes the first enzyme in the N-linked oligosaccharide processing pathway. The enzyme cleaves the distal alpha-1,2-linked glucose residue from the Glc(3)-Man(9)-GlcNAc(2) oligosaccharide precursor. This protein is located in the lumen of the endoplasmic reticulum. Defects in this gene are a cause of type IIb congenital disorder of glycosylation (CDGIIb). Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq
Specifications
Specifications
| Antigen | GCS1 |
| Applications | ELISA, Western Blot |
| Classification | Polyclonal |
| Conjugate | Unconjugated |
| Description | Rabbit polyclonal antibody raised against synthetic peptide of GCS1. |
| Dilution | ELISA (1:1000) Western Blot (1:100-500) The optimal working dilution should be determined by the end user. |
| Formulation | In PBS (0.09% sodium azide) |
| Gene | GCS1 |
| Gene Accession No. | GCS1_HUMAN |
| Gene Symbols | GCS1 |
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