Thermo Fisher Scientific GAA Monoclonal Antibody (2G7)
인간 GAA 단백질을 인식하는 Mouse IgG2b 단일클론 항체로 Western blot, IHC, ICC/IF에 사용 가능. 합성 펩타이드(494-527aa)를 면역원으로 제작. 동결건조 형태, 500 µg/mL 농도, 친화 크로마토그래피로 정제. 연구용으로만 사용.
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Applications
| Application | Tested Dilution |
|---|---|
| Western Blot (WB) | 0.1–0.5 µg/mL |
| Immunohistochemistry (Paraffin) (IHC (P)) | 0.5–1 µg/mL |
| Immunocytochemistry (ICC/IF) | 2 µg/mL |
Product Specifications
| Specification | Description |
|---|---|
| Species Reactivity | Human |
| Host / Isotype | Mouse / IgG2b |
| Class | Monoclonal |
| Type | Antibody |
| Clone | 2G7 |
| Immunogen | A synthetic peptide corresponding to a sequence in the middle region of human GAA (494–527aa TALAWWEDMVAEFHDQVPFDGMWIDMNEPSNFIR) |
| Conjugate | Unconjugated |
| Form | Lyophilized |
| Concentration | 500 µg/mL |
| Purification | Affinity chromatography |
| Storage Buffer | PBS with 4 mg trehalose |
| Contains | 0.01 mg 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_2884062 |
Product Specific Information
Reconstitute with 0.2 mL of distilled water to yield a concentration of 500 µg/mL.
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 are the cause of 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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