Anti-ARSA Rabbit Polyclonal Antibody
Product Information
Downloads & Supports
Target
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ARSA
Host
Storage
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Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
Clonality
Clone Number
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-
Isotype
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IgG
Reactivity
Application
Application Details
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WB: 1/500-1/1000 IHC: 1/50-1/200
Immunogen
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Synthetic peptide corresponding to Human ARSA.
Molecular Weight
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Calculated MW: 54kDa
Formulation
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1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2.
Form
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Liquid
Gene ID
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410
Uniprot ID
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P15289
Background
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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.
Research Area
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Synonyms
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Arylsulfatase A; ASA; Cerebroside-sulfatase; Arylsulfatase A component B; Arylsulfatase A component C; ARSA