| Category: |
Antibody |
| Concentration: |
2ml sterile DI water, 5mg/ml if reconstituted with 0 |
| Form: |
Antigen affinity purified |
| Conjugation: |
Unconjugated |
| Clone: |
Polyclonal antibody |
| Recognised antigen: |
GAA / Glucosidase alpha acid |
| Host animal: |
Rabbit (Oryctolagus cuniculus) |
| Clonality: |
Polyclonal (rabbit origin) |
| Species reactivity: |
Due to limited knowledge and inability to test the antibody against all known species, Rat , we cannot guarantee that no other cross reactivity can occur, Human (Homo sapiens) |
| Tested applications: |
IHC-P, WB |
| Recommended dilutions: |
5-1ug/ml, Immunohistochemistry (FFPE): 1-2ug/ml, Western blot: 0 |
| Notes: |
Optimal dilution of the GAA antibody should be determined by the researcher |
| Added buffer: |
025% sodium azide, 5% BSA and 0, Lyophilized from 1X Phosphate Buffered Saline (PBS) with 2 |
| Intented use: |
This GAA antibodyis to be used only for research purposes and not for diagnostics |
| Uniprot #: |
P10253 |
| Purity: |
Antigen affinity |
| Description: |
Alternative splicing results in multiple transcript variants, Defects in this gene are the cause of glycogen storage disease II, The encoded preproprotein is proteolytically processed to generate multiple intermediate forms and the mature form of the enzyme, This gene encodes lysosomal alpha-glucosidase, also known as Pompe's disease, which is an autosomal recessive disorder with a broad clinical spectrum, which is essential for the degradation of glycogen to glucose in lysosomes, Lysosomal alpha-glucosidase is an enzyme that in humans is encoded by the GAA gene |
| Immunogen: |
Amino acids 494-527 (TALAWWEDMVAEFHDQVPFDGMWIDMNEPSNFIR-human) were used as the immunogen for the GAA antibody |
| Storage: |
Avoid repeated freezing and thawing, Celcius or lower, Cycles of freezing and thawing can denaturate the peptide chains of the antibodies and reduce their sensitivity and/or change their affinity, Prepare aliqotes in such a manner so that freeze-thaw cycles are minimized, aliquot and store at -20 deg, the GAA antibody may be kept for up to one month refrigerated at +4 degrees C, After reconstitution, For long-term |
| Localization: |
membranous, Cytoplasmic |
| Properties: |
C, C for long term storage and for short term at + 5°, If you buy Antibodies supplied by NJS poly they should be stored frozen at - 24° |
| Gene target: |
GAA / Glucosidase alpha acid |
| Short name: |
GAA Antibody / Glucosidase alpha acid |
| Technique: |
antibodies against human proteins, antibodies for, Antibody |
| Alternative name: |
GAA (Antibody to) / Glucosidase a acid |
| Alternative technique: |
antibodies |
| Identity: |
4065 |
| Gene: |
GAA |
More about : GAA |
| Long gene name: |
acid , glucosidase alpha |
| Synonyms gene name: |
acid , alpha, glucosidase |
| Synonyms name: |
Pompe disease glycogen storage disease type II |
| Locus: |
17q25, 3 |
| Discovery year: |
1986-01-01 |
| Entrez gene record: |
2548 |
| Havana BLAST/BLAT: |
OTTHUMG00000177537 |
| Locus Specific Databases: |
GAA - Pompe disease (glycogen storage disease type II) LOVD - Leiden Open Variation Database LRG_673 |