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

More general terms

• arylsulfatase
• lysosomal hydrolase

Database

• UniProt: http://www.uniprot.org/uniprot/P15289.html
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:410
• OMIM: https://mirror.omim.org/entry/250100
• OMIM: https://mirror.omim.org/entry/272200
• OMIM: https://mirror.omim.org/entry/607574
• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=410