Thermo Fisher Scientific GCS1 Polyclonal Antibody
Thermo Fisher Scientific의 GCS1 Polyclonal Antibody는 인간 GCS1 단백질을 인식하는 Rabbit IgG 항체입니다. Western blot과 ICC/IF에 적합하며, 항원 친화 크로마토그래피로 정제되었습니다. PBS/glycerol 완충액에 보관되며 연구용으로만 사용 가능합니다.
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Applications
Western Blot (WB)
- Tested Dilution: 0.04–0.4 µg/mL
Immunocytochemistry (ICC/IF)
- Tested Dilution: 0.25–2 µg/mL
Product Specifications
| 항목 | 내용 |
|---|---|
| Species Reactivity | Human |
| Host / Isotype | Rabbit / IgG |
| Class | Polyclonal |
| Type | Antibody |
| Immunogen | Recombinant Human GCS1. Recombinant protein control fragment (Product # RP-90142) |
| Conjugate | Unconjugated |
| Form | Liquid |
| Concentration | 0.05 mg/mL |
| Purification | Antigen affinity chromatography |
| Storage buffer | PBS, pH 7.2, with 40% glycerol |
| Contains | 0.02% sodium azide |
| 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_2663261 |
Product Specific Information
Immunogen sequence:
LESHAEGFRER FEKTFQLKEK GLSSGEQVLG QAALSGLLGG IGYFYGQGLV LPDIGVEGSE QKVDPALFPP VPLFTAVPSR SFFPRGFLWD EGFHQLVVQR WDPSLTREAL GHWLGLLNAD GWIG
Highest antigen sequence identity to the following orthologs:
- Mouse: 89%
- Rat: 88%
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 may include hepatomegaly, hypoventilation, feeding problems, and seizures. The clinical course is progressive, with survival typically limited to a few months.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
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