Thermo Fisher Scientific GAA Recombinant Rabbit Monoclonal Antibody (ARC2392)
GAA 단백질을 인식하는 Recombinant Rabbit Monoclonal 항체로, Western blot 및 ELISA에 적합합니다. 인간 시료에 반응하며 HEK293 세포에서 발현되었습니다. 고순도 친화 크로마토그래피 정제, PBS/glycerol buffer 보존, 연구용 전용 제품입니다.
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Applications
Western Blot (WB)
- Tested Dilution: 1:1,000–1:6,000
ELISA
- Tested Dilution: 1 µg/mL
Product Specifications
| 항목 | 내용 |
|---|---|
| Species Reactivity | Human |
| Host / Isotype | Rabbit / IgG |
| Expression System | HEK293 cells |
| Class | Recombinant Monoclonal |
| Type | Antibody |
| Clone | ARC2392 |
| Immunogen | A synthetic peptide corresponding to a sequence within amino acids 100–200 of human GAA (P10253) |
| Conjugate | Unconjugated |
| Form | Liquid |
| Concentration | 0.8 mg/mL |
| Purification | Affinity chromatography |
| Storage Buffer | PBS, pH 7.3, with 50% glycerol, 0.05% BSA |
| Contains | 0.02% sodium azide |
| Storage Conditions | -20°C, Avoid Freeze/Thaw Cycles |
| Shipping Conditions | Ambient (domestic); Wet ice (international) |
| RRID | AB_2897790 |
Product Specific Information
- Positive Samples: 293T, HepG2, DU145
- Immunogen sequence:
QEQCEARGCC YIPAKQGLQG AQMGQPWCFF PPSYPSYKLE NLSSSEMGYT ATLTRTTPTF FPKDILTLRL DVMMETENRL HFTIKDPANR RYEVPLETPHV
Target Information
The GAA (acid alpha-glucosidase) gene encodes an enzyme essential for glycogen degradation to glucose in lysosomes.
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 identified.
For Research Use Only.
Not for use in diagnostic procedures.
Not for resale without express authorization.
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