Monoclonal Anti-GAA antibody produced in mouse
SIGMA/WH0002548M1 - clone 3C6, purified immunoglobulin, buffered aqueous solution
Synonym: Anti-LYAG; Anti-glucosidase, α acid (Pompe disease, glycogen storage disease type II)
Product Type: Chemical
Western Blotting QC Western Blot detection against Immunogen (36.96 kDa).
ELISA Detection limit for recombinant GST tagged GAA is 0.3 ng/mL as a capture antibody.
| antibody form | purified immunoglobulin |
| antibody product type | primary antibodies |
| biological source | mouse |
| clone | 3C6, monoclonal |
| conjugate | unconjugated |
| form | buffered aqueous solution |
| GenBank accession no. | BC040431   |
| isotype | IgG1κ |
| Quality Level | 100 |
| shipped in | dry ice |
| species reactivity | human |
| storage temp. | −20°C |
| target post-translational modification | unmodified |
| technique(s) | indirect ELISA: suitable |
| western blot: 1-5 μg/mL | |
| UniProt accession no. | P10253 |
| Biochem/physiol Actions: | Lysosomal α-glucosidase (GAA) uses mannose-6-phosphate receptors for its localization on the lysosomes. It mediates the hydrolysis of glycogen to glucose. Mutations in the GAA gene impair acid alpha-glucosidase enzyme activity. Deficiency of GAA leads to a rare lysosomal storage disease namely Pompe disease. |
| Disclaimer: | Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals. |
| General description: | Lysosomal α-glucosidase (GAA), a glycoprotein and member of glycoside hydrolase family GH31, comprises a trefoil type-P domain, catalytic GH31 domain, distal, proximal, and an N-terminal β-sheet domain. The GAA gene is mapped to human chromosome 17q25.3. It undergoes various proteolytical and N-glycan processing in the late endosomal/lysosomal compartment to become an active form. |
| Immunogen: | GAA (AAH40431, 851 a.a. ~ 952 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa. Sequence GEARGELFWDDGESLEVLERGAYTQ |
| Legal Information: | GenBank is a registered trademark of United States Department of Health and Human Services |
| Physical form: | Solution in phosphate buffered saline, pH 7.4 |
| RIDADR | NONH for all modes of transport |
| Flash Point(F) | Not applicable |
| Flash Point(C) | Not applicable |
| Storage Temp. | −20°C |
| UNSPSC | 12352203 |