Mannosyl-Oligosaccharide Glucosidase (GCS1) Antibody
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Catalogue No: abx031580
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US$326.25
(Size: 80 µl)
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Datasheet SDS
GCS1 cleaves the distal alpha 1, 2-linked glucose residue from the Glc (3) Man (9) GlcNAc (2) oligosaccharide precursor in a highly specific manner. Defects in GCS1 are the cause of type IIb congenital disorder of glycosylation (CDGIIb). This syndrome is also known as glucosidase I deficiency and is characterized by marked generalized hypotonia and hypomotility of the neonate, dysmorphic features, including a prominent occiput, short palpebral fissures, retrognathia, high arched palate, generalized edema, and hypoplastic genitalia. Symptoms include hepatomegaly, hypoventilation, feeding problems and seizures. The clinical course is progressive and survival is at most a few months.
Research Articles on Mannosyl-Oligosaccharide Glucosidase (GCS1)
| Target | Mannosyl-Oligosaccharide Glucosidase (GCS1) |
| Clonality | Polyclonal |
| Reactivity | Human |
| Tested Applications | ELISA, WB |
| Host | Rabbit |
| Recommended dilutions | WB: 1/1000. Optimal dilutions/concentrations should be determined by the end user. |
| Conjugation | Unconjugated |
| Immunogen | KLH-conjugated synthetic peptide between 1-30 amino acids from the N-terminal region of human GCS1. |
| Isotype | IgG |
| Form | Liquid |
| Purification | Purified through a protein G column, eluted with high and low pH buffers and neutralized immediately, followed by dialysis against PBS. |
| Storage | Aliquot and store at 2-8°C for up to 2 weeks, or at -20°C for long term storage. Avoid repeated freeze/thaw cycles. |
| Shelf Life | 12 months. |
| UniProt Primary AC | Q13724 (UniProt, ExPASy) |
| UniProt Secondary AC | A8K938, F5H6D0, Q17RN9, Q8TCT5 |
| UniProt Entry Name | MOGS_HUMAN |
| KEGG | hsa:7841 |
| String | 9606.ENSP00000233616 |
| Molecular Weight | Calculated MW: 91.9 kDa |
| Buffer | PBS containing 0.09% sodium azide. |
| Availability | Shipped within 5-10 working days. |
| Note | THIS PRODUCT IS FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC, THERAPEUTIC OR COSMETIC PROCEDURES. NOT FOR HUMAN OR ANIMAL CONSUMPTION. |