Orphanet: Mucopolysaccharidosis type 6

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Mucopolysaccharidosis type 6

Disease definition

Mucopolysaccharidosis type 6 (MPS 6) is a lysosomal storage disease with progressive multisystem involvement, associated with a deficiency of arylsulfatase B (ASB) leading to the accumulation of dermatan sulfate.


Classification level: Disorder
  • Synonym(s):
    • ARSB deficiency
    • ASB deficiency
    • Arylsulfatase B deficiency
    • MPS6
    • MPSVI
    • Maroteaux-Lamy disease
    • Mucopolysaccharidosis type VI
    • N-acetylgalactosamine 4-sulfatase deficiency
  • Prevalence: 1-9 / 1 000 000
  • Inheritance: Autosomal recessive 
  • Age of onset: Childhood
  • ICD-10: E76.2
  • OMIM: 253200
  • UMLS: C0026709
  • MeSH: D009087
  • GARD: 7095
  • MedDRA: 10056892

Detailed information


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