Thermo Fisher Scientific GCS1 Polyclonal Antibody
Rabbit polyclonal antibody against Human GCS1 for Western blot applications. Recognizes recombinant fragment (aa 136–355) of Glucosidase I. Antigen affinity purified, 1 mg/mL liquid form, unconjugated. Suitable for 293T, HeLa, HepG2, and related cell l...
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Applications
Western Blot (WB)
- Tested Dilution: 1:500–1:3,000
Product Specifications
| 항목 | 내용 |
|---|---|
| Species Reactivity | Human |
| Host / Isotype | Rabbit / IgG |
| Class | Polyclonal |
| Type | Antibody |
| Immunogen | Recombinant fragment corresponding to a region within amino acids 136–355 of Human Glucosidase I |
| Conjugate | Unconjugated |
| Form | Liquid |
| Concentration | 1 mg/mL |
| Purification | Antigen affinity chromatography |
| Storage Buffer | 0.1M Tris glycine, pH 7, with 10% glycerol |
| Contains | 0.01% thimerosal |
| Storage Conditions | Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles. |
| Shipping Conditions | Wet ice |
| RRID | AB_2545788 |
Product Specific Information
- Recommended positive controls: 293T, A431, H1299, HeLaS3, HepG2, Molt-4, Raji
- Predicted reactivity: Mouse (87%)
- Store product as a concentrated solution. Centrifuge briefly prior to opening the vial.
Target Information
GCS1 cleaves the distal α1,2-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), also known as glucosidase I deficiency.
This syndrome is characterized by generalized hypotonia, hypomotility, dysmorphic features (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, with survival typically limited to a few months.
⚠ WARNING: This product can expose you to chemicals including mercury, which is known to the State of California to cause birth defects or other reproductive harm.
For more information, visit www.P65Warnings.ca.gov.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
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