Product Specifications | |
---|---|
Species Reactivity |
Human |
Host/Isotype |
Mouse / IgG2b |
Class |
Monoclonal |
Type |
Antibody |
Clone |
2G7 |
Immunogen |
A synthetic peptide corresponding to residues 494-527 of human GAA (TALAWWEDMV AEFHDQVPFD GMWIDMNEPS NFIR), different from the related mouse sequence by eight amino acids, and from the related rat sequence by six amino acids |
Conjugate |
Unconjugated |
Form |
Lyophilized |
Concentration |
500 µg/mL |
Purification |
Affinity chromatography |
Storage buffer |
PBS with 4mg trehalose |
Contains |
0.05mg sodium azide |
Storage conditions |
Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles. |
RRID |
AB_2884062 |
Add 0.2 mL of distilled water will yield a concentration of 500 µg/mL.
Sequence of this protein is as follows: TALAWWEDMV AEFHDQVPFD GMWIDMNEPS NFIR.
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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
Protein Aliases: 70 kDa lysosomal alpha-glucosidase; 76 kDa lysosomal alpha-glucosidase; Acid maltase; Aglucosidase alfa; Lysosomal alpha-glucosidase
Gene Aliases: GAA; LYAG
UniProt ID: (Human) P10253
Entrez Gene ID: (Human) 2548
Molecular Function:
glucosidase
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