Thermo Fisher Scientific GAA Monoclonal Antibody (3C6)
인간 GAA 단백질을 인식하는 마우스 단클론 항체로 Western blot 및 ELISA에 적합. 고순도 친화 크로마토그래피 정제. PBS 저장용액, 방부제 무첨가. 연구용으로만 사용 가능.
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Applications
Western Blot (WB)
- Tested Dilution: 1–5 µg/mL
ELISA
- Tested Dilution: 0.3 ng/mL
Product Specifications
| 항목 | 내용 |
|---|---|
| Species Reactivity | Human |
| Host / Isotype | Mouse / IgG1, kappa |
| Class | Monoclonal |
| Type | Antibody |
| Clone | 3C6 |
| Immunogen | GAA (AAH40431, 851–952 a.a.) partial recombinant protein with GST tag (MW of GST tag: 26 kDa) |
| Conjugate | Unconjugated |
| Form | Liquid |
| Concentration | See Label |
| Purification | Affinity chromatography |
| Storage Buffer | PBS, pH 7.4 |
| Contains | No preservative |
| Storage Conditions | -20°C, Avoid Freeze/Thaw Cycles |
| Shipping Conditions | Ambient (domestic); Wet ice (international) |
Product Specific Information
Sequence of this protein is as follows:
GEARGELFWD DGESLEVLER GAYTQVIFLA RNNTIVNELV RVTSEGAGLQ LQKVTVLGVA TAPQQVLSNG VPVSNFTYSP DTKVLDICVS LLMGEQFLVS WC
Target Information
This gene encodes acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing.
Defects in this gene cause glycogen storage disease II (Pompe’s disease), an autosomal recessive disorder with a broad clinical spectrum.
Three transcript variants encoding the same protein have been found for this gene.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
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