ITT0347
ITT0347
- Catalog: ITT0347
- Gene/Protein: ARSA
- Product Description: Immunotag™ Arylsulfatase A Polyclonal Antibody
385.0000
Price in reward points: 385
| Antibody Specification | |
| Datasheet | 
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| Target Protein | Arylsulfatase A |
| Clonality | Polyclonal |
| Storage/Stability | -20°C/1 year |
| Application | WB,IHC-p,ELISA |
| Recommended Dilution | Western Blot: 1/500 - 1/2000. Immunohistochemistry: 1/100 - 1/300. ELISA: 1/20000. Not yet tested in other applications. |
| Concentration | 1 mg/ml |
| Reactive Species | Human,Mouse,Rat |
| Host Species | Rabbit |
| Immunogen | The antiserum was produced against synthesized peptide derived from human ARSA. AA range:251-300 |
| Specificity | Arylsulfatase A Polyclonal Antibody detects endogenous levels of Arylsulfatase A protein. |
| Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen |
| Form | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Gene Name | ARSA |
| Accession No. | P15289 P50428 |
| Alternate Names | ARSA; Arylsulfatase A; ASA; Cerebroside-sulfatase |
| Description | arylsulfatase A(ARSA) Homo sapiens The protein encoded by this gene hydrolyzes cerebroside sulfate to cerebroside and sulfate. Defects in this gene lead to metachromatic leucodystrophy (MLD), a progressive demyelination disease which results in a variety of neurological symptoms and ultimately death. Alternatively spliced transcript variants have been described for this gene. [provided by RefSeq, Dec 2010], |
| Cell Pathway/ Category | Sphingolipid metabolism,Lysosome, |
| Protein Expression | B-cell,Liver,Small intestine,Testis, |
| Subcellular Localization | acrosomal vesicle,extracellular space,lysosome,endosome,endoplasmic reticulum lumen,integral component of membrane,extrinsic component of external side of plasma membrane,lysosomal lumen,extracellular exosome, |
| Protein Function | catalytic activity:A cerebroside 3-sulfate + H(2)O = a cerebroside + sulfate.,cofactor:Binds 1 magnesium ion per subunit.,disease:Arylsulfatase A activity is defective in multiple sulfatase deficiency (MSD) [MIM:272200]. MSD is a disorder characterized by decreased activity of all known sulfatases. MSD is due to defects in SUMF1 resulting in the lack of post-translational modification of a highly conserved cysteine into 3-oxoalanine. It combines features of individual sulfatase deficiencies such as metachromatic leukodystrophy, mucopolysaccharidosis, chondrodysplasia punctata, hydrocephalus, ichthyosis, neurologic deterioration and developmental delay.,disease:Defects in ARSA are a cause of leukodystrophy metachromatic (MLD) [MIM:250100]. MLD is a disease due to a lysosomal storage defect. It is characterized by intralysosomal storage of cerebroside-3-sulfate in neural and non-neural tissues, with a diffuse loss of myelin in the central nervous system. Progressive demyelination causes a variety of neurological symptoms, including gait disturbances, ataxias, optical atrophy, dementia, seizures, and spastic tetraparesis. Three forms of the disease can be distinguished according to the age at onset: late-infantile, juvenile and adult.,function:Hydrolyzes cerebroside sulfate.,online information:Arylsulfatase A entry,PTM:The conversion to 3-oxoalanine (also known as C-formylglycine, FGly), of a serine or cysteine residue in prokaryotes and of a cysteine residue in eukaryotes, is critical for catalytic activity. This post-translational modification is severely defective in multiple sulfatase deficiency (MSD).,similarity:Belongs to the sulfatase family.,subunit:Homodimer at neutral pH and homooctamer at acidic pH. Exists both as a single chain of 58 kDa (component A) or as a chain of 50 kDa (component B) linked by disulfide bond(s) to a 7 kDa chain (component C)., |
| Usage | For Research Use Only! Not for diagnostic or therapeutic procedures. |
| Material Safety Data Sheet | |
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