Target:Arylsulfatase A
Fields:Sphingolipid metabolism;Metabolic pathways;Lysosome
Gene Name:ARSA
Protein Name:Arylsulfatase A
Human Gene Id:410
Human Swiss Prot No:P15289
Mouse Gene Id:11883
Mouse Swiss Prot No:P50428
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.
Formulation:Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Source:Polyclonal, Rabbit,IgG
Dilution:WB 1:500 - 1:2000. IHC 1:100 - 1:300. ELISA: 1:20000.. IF 1:50-200
Purification:The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Concentration:1 mg/ml
Storage Stability:-15°C to -25°C/1 year(Do not lower than -25°C)
Other Name:ARSA;Arylsulfatase A;ASA;Cerebroside-sulfatase
Observed Band(KD):54kD
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],
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 tis
Subcellular Location:Endoplasmic reticulum . Lysosome .
Expression: B-cell,Liver,Small intestine,Testis,