BS3077

antibody from Bioworld Technology, Inc

Targeting: ARSA

Immunohistochemistry

Antibody data

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Validation data

Product number: BS3077 - Provider product page
Provider: Bioworld Technology, Inc
Product name: ARSA (R291) polyclonal antibody
Antibody type: Polyclonal
Antigen: Synthetic peptide, corresponding to amino acids 260-310 of Human ARSA.
Description: 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.
Reactivity: Human, Mouse, Rat
Host: Rabbit
Isotype: IgG
Vial size: 100ul
Concentration: 1 mg/ml
Storage: Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.

ARSA protein structure - BS3077 shown in red.