Rabbit Anti-Human GAA (N-term) Antibody, 400 ul
Loading...
Loading...
Loading...
Loading...
Product Description
Specifications
| Size | 400 µL |
| Estimated Lead Time | 1-2 weeks |
| Species | Human |
| Host Species | Rabbit |
| Accession Number | P10253 |
| Gene Id | 2548 |
| Gene Symbols | GAA |
| Protein Name / Synonyms | GAA (N-term) |
| Clonality | Polyclonal |
| Preparation | This GAA antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 174-203 amino acids from the N-terminal region of human GAA. |
| Concentration (lot specific) | 0.460 mg/ml |
| Recommended Applications | Western Blotting, IHC-P |
| Research Area | Cardiovascular Disease |
| Shipping Type | Blue ice |
| Storage | -20°C |
Introduction
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, also known as Pompe's disease, which is an autosomal recessive disorder with a broad clinical spectrum. Three transcript variants encoding the same protein have been found for this gene.
Specificity
Other Names
GAA; Lysosomal alpha-glucosidase; Acid maltase; Aglucosidase alfa; 76 kDa lysosomal alpha-glucosidase; 70 kDa lysosomal alpha-glucosidase
NCBI Accession #
NP_000143.2;NP_001073271.1;NP_001073272.1
Other Accession #
NP_000143.2; NP_001073271.1
Format
Type
Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)
Calculated Molecular Weight (Da)
105324
Recommended Dilutions
WB: 1:1000; IHC: 1:10-50
 | GAA Antibody (N-term) (Cat# 102-11622) western blot analysis in HL-60 cell line lysates (35ug/lane).This demonstrates the GAA antibody detected the GAA protein (arrow). |
 | GAA Antibody (N-term) (Cat# 102-11622)immunohistochemistry analysis in formalin fixed and paraffin embedded human lung carcinoma followed by peroxidase conjugation of the secondary antibody and DAB staining.This data demonstrates the use of GAA Antibody (N-term) for immunohistochemistry. Clinical relevance has not been evaluated. |
Antigen Source
HUMAN
Storage/Stability
2-8°C (short-term); -20°C (long-term)
Expiration:
12 months from the date of shipment when stored properly.