- Please choose product options by visiting U-Blot® Mouse ZP2 Antibody (IE-3/CRL-2463), APC.
Arylsulfatase A Polyclonal Antibody
Ucallm_WP11147-50
Price range: $268.00 through $328.00
Product details
Background:
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],
Specifications
| Size | 50μL/100μL |
| Target | Arylsulfatase A |
| Reactivity | Human, Mouse, Rat |
| Applications | WB, IHC, IF, ELISA |
| MW(Calculated) | |
| MW(Observed) | 54kD |
| Host Species | Rabbit |
| Isotype | IgG |
| Conjugate/Modification | Unmodified |
| Recommended Dilution Ratio | WB 1:500-1:2000; IHC 1:100-1:300; ELISA 1:20000; IF 1:50-200 / Note: For IHC, we suggest antigen retrieval with TE buffer pH 9.0 (Cat#WT1004) |
| Formulation | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen. |
| Storage | -15°C to -25°C/1 year(Do not lower than -25°C) |
| Concentration | 1 mg/ml |
| Clonality | Polyclonal |
| Clone Number | |
| 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. |
| Gene Name | ARSA |
| Protein Name | Arylsulfatase A |
| Other Name | ARSA; / Arylsulfatase A; / ASA; / Cerebroside-sulfatase |
| Species | Human |
| Gene ID | 410 |
| Uniprot | P15289, |
| Species.1 | Mouse |
| Gene ID.1 | 11883 |
| Uniprot.1 | P50428 |
| Species.2 | |
| Gene ID.2 | |
| Uniprot.2 | |
| Cellular Localization | Endoplasmic reticulum . Lysosome . |
| Tissue Expression | B-cell,Liver,Small intestine,Testis, |
| Signaling_pathway | Cellular Processes >> Transport and catabolism >> Lysosome |
| Research Areas | >>Sphingolipid metabolism; / >>Metabolic pathways; / >>Lysosome |
| 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)., |
Please login to post questions
Related products
