Application
Immunofluorescence staining of methanol-fixed untransfected (A) and human ASA transfected HEK 293T cells (B).Application
Western blot analysis of ASA expression in non-transfected: (A) and human ASA transfected: (B) 293T whole cell lysates.Application
Western blot analysis of ASA expression in non-transfected: (A) and mouse ASA transfected: (B) 293T whole cell lysates.Application
Western blot analysis of ASA expression in mouse liver tissue extract.| Product Name | ASA Antibody (H-129) |
|---|---|
| Antibody Type | Primary Antibodies |
| Antigen Alias | epitope corresponding to amino acids 145-273 mapping within an internal region of ASA of human origin |
| Modification Notes | ASA (arylsulfatase A), also known as Cerebroside-sulfatase, ARSA or MLD, is a 507 amino acid lysosomal protein that belongs to the sulfatase family. Functioning as a homodimer at a neutral pH and as a homooctamer at an acidic pH, ASA uses magnesium as a cofactor to catalyze the H2O-dependent hydrolysis of cerebroside 3-sulfate to cerebroside and sulfate. Defects in the gene encoding ASA are a cause of metachromatic leukodystrophy (MLD), an intralysosomal storage disease that is characterized by ataxias, dementia, seizures, spastic tetraparesis and, ultimately, death. Additionally, defects in ASA activity are associated with multiple sulfatase deficiency (MSD), a disorder that results in decreased activity of all known sulfatases and is generally characterized by metachromatic leukodystrophy, mucopolysaccharidosis, chondrodysplasia punctata, hydrocephalus, ichthyosis, neurologic deterioration and developmental delay. |
| Clonality | Polyclonal |
|---|---|
| Isotype | IgG |
| Host Species | Rabbit |
| Tested Applications | WBIPIFELISA |
| recommended for detection of ASA of mouse, rat and human origin by WB, IP, IF and ELISA also reactive with additional species, including equine, canine, bovine and porcine: |
|
| Species Reactivity | HumanMouseRat |
| Concentration | 1mg/ml |
| Purification | Affinity purified |
| Alternative Names | epitope corresponding to amino acids 145-273 mapping within an internal region of ASA of human origin |
|---|---|
| Tissue Specificity | epitope corresponding to amino acids 145-273 mapping within an internal region of ASA of human origin |
| Entrez Gene | 410 |
|---|
Application
Immunofluorescence staining of methanol-fixed untransfected (A) and human ASA transfected HEK 293T cells (B).
Application
Western blot analysis of ASA expression in non-transfected: (A) and human ASA transfected: (B) 293T whole cell lysates.
Application
Western blot analysis of ASA expression in non-transfected: (A) and mouse ASA transfected: (B) 293T whole cell lysates.
Application
Western blot analysis of ASA expression in mouse liver tissue extract.| Application Notes | recommended for detection of ASA of mouse, rat and human origin by WB, IP, IF and ELISA also reactive with additional species, including equine, canine, bovine 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. |
