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ARSA (R291) pAb

ARSA (R291) pAb

Cat no: BS3077

Supplier: Bioworld Technology, Inc.
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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.
Catalogue number: BS3077
Reactivities: Human, Mouse, Rat
Hosts: Rabbit
Applications: Immunohistochemistry, Western Blot
Size: 100ug/100ul
Swiss prot: P15289
Dilutions: WB: 1:500~1:1000 IHC: 1:50~1:200
Form: liquid
Storage buffer: 1 mg/ml in Phosphate buffered saline (PBS) with 0.05% sodium azide, approx. pH 7.2.
Concentration: 1ug/ul
Antigen: Synthetic peptide, corresponding to amino acids 260-310 of Human ARSA.
Species: ARSA (R291) pAb detects endogenous levels of ARSA protein.
Molecular weight: ~ 54 kDa
Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).
Storage temp: Store at 4 degrees C short term. Aliquot and store at -20 degrees C long term. Avoid freeze-thaw cycles.
Alternative names: Arylsulfatase A; ASA; Cerebroside-sulfatase; Arylsulfatase A component B; Arylsulfatase A component C; ARSA
Additional info: For research use only, not for use in diagnostic procedure.

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