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Literature summary for 3.1.6.12 extracted from

  • Harmatz, P.; Giugliani, R.; Schwartz, I.V.; Guffon, N.; Teles, E.L.; Miranda, M.C.; Wraith, J.E.; Beck, M.; Arash, L.; Scarpa, M.; Ketteridge, D.; Hopwood, J.J.; Plecko, B.; Steiner, R.; Whitley, C.B.; Kaplan, P.; Yu, Z.F.; Swiedler, S.J.; Decker, C.; Decker, C.
    Long-term follow-up of endurance and safety outcomes during enzyme replacement therapy for mucopolysaccharidosis VI: Final results of three clinical studies of recombinant human N-acetylgalactosamine 4-sulfatase (2008), Mol. Genet. Metab., 94, 469-475.
    View publication on PubMed

Application

Application Comment Organism
medicine mucopolysaccharidosis type VI is a lysosomal storage disease in which deficient activity of the enzyme arylsulfatase B impairs the stepwise degradation of the glycosaminoglycan dermatan sulfate Homo sapiens

Cloned(Commentary)

Cloned (Comment) Organism
expressed in Escherichia coli Homo sapiens

Localization

Localization Comment Organism GeneOntology No. Textmining
lysosome
-
Homo sapiens 5764
-

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
dermatan sulfate + H2O
-
Homo sapiens dermatan + sulfate
-
?

Synonyms

Synonyms Comment Organism
arylsulfatase B
-
Homo sapiens
ASB
-
Homo sapiens
N-acetylgalactosamine 4-sulfatase
-
Homo sapiens