Application
Immunoperoxidase staining of formalin fixed, paraffin-embedded human pancreas tissue showing cytoplasmic staining of Islets of Langerhans.Application
Western blot analysis of α-gal A expression in non-transfected: (A) and human α-gal A transfected: (B) 293T whole cell lysates.| Product Name | α-gal A Antibody (H-104) |
|---|---|
| Antibody Type | Primary Antibodies |
| Antigen Alias | epitope corresponding to amino acids 326-429 mapping at the C-terminus of α-galactosidase A of human origin |
| Modification Notes | å-galactosidase A (å-gal A) functions as a lysosomal hydrolase. å-gal A forms an active homodimer that acts upon a glycolipid substrate, globotriaosylceramide (Gb3). The gene encoding å-gal A maps to chromosome Xq22 (1). Inherited mutations in this gene cause an X-linked recessive glycolipid storage disorder known as Fabry’s disease (2). In Fabry patients, å-gal A deficiencies lead to an accumulation of Gb3 in the body. The numerous clinical manifestations of the disease include renal and cardiac impairment, severe pain in the extremities and cutaneous lesions known as angiokeratomas. Enzyme replacement therapy using recombinant å-gal A effectively treats the symptoms of Fabry disease (3-6). |
| Clonality | Polyclonal |
|---|---|
| Isotype | IgG |
| Host Species | Rabbit |
| Tested Applications | WBIPIFIHCELISA |
| recommended for detection of α-gal A of human and, to a lesser extent, mouse and rat origin by WB, IP, IF, IHC(P) and ELISA: |
|
| Species Reactivity | HumanMouseRat |
| Concentration | 1mg/ml |
| Purification | Affinity purified |
| Alternative Names | epitope corresponding to amino acids 326-429 mapping at the C-terminus of &alpha -galactosidase A of human origin |
|---|---|
| Tissue Specificity | epitope corresponding to amino acids 326-429 mapping at the C-terminus of α-galactosidase A of human origin |
| Entrez Gene | 2717 |
|---|
Application
Immunoperoxidase staining of formalin fixed, paraffin-embedded human pancreas tissue showing cytoplasmic staining of Islets of Langerhans.
Application
Western blot analysis of α-gal A expression in non-transfected: (A) and human α-gal A transfected: (B) 293T whole cell lysates.| Application Notes | recommended for detection of α-gal A of human and, to a lesser extent, mouse and rat origin by WB, IP, IF, IHC(P) and ELISA: |
|---|
| 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. |
