Application
Western blot analysis of AGA expression in non-transfected: (A) and human AGA transfected: (B) 293T whole cell lysates.| Product Name | AGA Antibody (H-300) |
|---|---|
| Antibody Type | Primary Antibodies |
| Antigen Alias | epitope corresponding to amino acids 47-272 mapping at the C-terminus of AGA of human origin |
| Modification Notes | AGA (aspartylglucosaminidase) is a 346 amino acid precursor protein that belongs to the Ntn-hydrolase family and is cleaved to produce an alpha chain and a beta chain. Localized to the lysosome, AGA functions as a heterotetramer composed of two alpha and two beta chains that work together to cleave the GlcNAc-Asn bond that joins oligosaccharides to target glycoproteins. Defects in the gene encoding AGA are the cause of aspartylglucosaminuria (AGU), a lysosomal storage disease that is characterized by severe mental retardation and mild connective tissue abnormalities. The gene encoding AGA maps to human chromosome 4, which encodes nearly 6% of the human genome and has the largest gene deserts (regions of the genome with no protein encoding genes) of all of the human chromosomes. |
| Clonality | Polyclonal |
|---|---|
| Isotype | IgG |
| Host Species | Rabbit |
| Tested Applications | WBIPIFELISA |
| recommended for detection of AGA of mouse, rat and human origin by WB, IP, IF and ELISA also reactive with additional species, including canine and porcine: |
|
| Species Reactivity | HumanMouseRat |
| Concentration | 1mg/ml |
| Purification | Affinity purified |
| Alternative Names | epitope corresponding to amino acids 47-272 mapping at the C-terminus of AGA of human origin |
|---|---|
| Tissue Specificity | epitope corresponding to amino acids 47-272 mapping at the C-terminus of AGA of human origin |
| Entrez Gene | 175 |
|---|
Application
Western blot analysis of AGA expression in non-transfected: (A) and human AGA transfected: (B) 293T whole cell lysates.| Application Notes | recommended for detection of AGA of mouse, rat and human origin by WB, IP, IF and ELISA also reactive with additional species, including canine and porcine: |
|---|
| Form | Liquid |
|---|---|
| Storage Instructions | For short-term storage, store at 4° C. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles. |
| Storage Buffer | phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol. |
