Search
arylsulfatase-A (cerebroside sulfatase, [contains: arylsulfatase A component B; arylsulfatase A component C], ARSA)
Function:
- hydrolyzes cerebroside sulfate
cerebroside 3-sulfate + H2O cerebroside + sulfate
Cofactor: binds 1 Mg+2 per subunit
Structure:
- homodimer at neutral pH & homooctamer at acidic pH
- exists both as a single chain of 58 kD (component A) or as a chain of 50 kD (component B) linked by disulfide bond(s) to a 7 kD chain (component C)
- belongs to the sulfatase family
Compartment: lysosome
Pathology:
- defects in ARSA are a cause of metachromatic leukodystrophy
- arylsulfatase A activity is defective in multiple sulfatase deficiency
Laboratory:
- ARSA gene mutation
- cerebroside sulfatase in leukocytes
Related
ARSA gene mutation
ARSA gene PD allele
cerebroside sulfatase in 24 hour urine
General
arylsulfatase
lysosomal hydrolase
Properties
SIZE: entity length = 507 aa
MW = 54 kD
COMPARTMENT: lysosome
MOTIF: signal sequence {1-18}
Mg+2-binding site
SITE: 29-29
Mg+2-binding site
SITE: 30-30
Mg+2-binding site
SITE: 69-69
histidine residue {H125}
cysteine residue {C156}
MODIFICATION: cysteine residue {C172}
N-glycosylation site {N158}
cysteine residue {C161}
MODIFICATION: cysteine residue {C168}
cysteine residue {C168}
MODIFICATION: cysteine residue {C161}
cysteine residue {C172}
MODIFICATION: cysteine residue {C156}
N-glycosylation site {N184}
Mg+2-binding site
SITE: 281-281
Mg+2-binding site
SITE: 282-282
cysteine residue {C300}
MODIFICATION: cysteine residue {C414}
N-glycosylation site {N350}
cysteine residue {C414}
MODIFICATION: cysteine residue {C300}
cysteine residue {C488}
MODIFICATION: cysteine residue {C500}
cysteine residue {C489}
MODIFICATION: cysteine residue {C502}
cysteine residue {C493}
MODIFICATION: cysteine residue {C499}
cysteine residue {C499}
MODIFICATION: cysteine residue {C493}
cysteine residue {C500}
MODIFICATION: cysteine residue {C488}
cysteine residue {C502}
MODIFICATION: cysteine residue {C489}
Database Correlations
OMIM correlations
MORBIDMAP 607574
UniProt P15289
Pfam PF00884
Entrez Gene 410
Kegg hsa:410
ENZYME 3.1.6.8
References
- OMIM :accession 250100
- GeneReviews
https://www.genecards.org/cgi-bin/carddisp.pl?gene=ARSA
- Wikipedia, arylsulfatase A entry
http://en.wikipedia.org/wiki/Arylsulfatase_A
- UniProt :accession P15289