FB2025_05 , released December 11, 2025
FB2025_05 , released December 11, 2025
CV Term Report
Help Close All Open All
General Information
Term mucopolysaccharidosis VI ID (Ontology) DOID:12800 (Human Disease)
Definition A mucopolysaccharidosis characterized by a deficiency of the lysosomal enzyme N-acetylgalactosamine 4-sulfatase.
Also Known As "arylsulfatase B deficiency" ; "deficiency of N-acetylgalactosamine-4-sulfatase" ; "Maroteaux - Lamy syndrome" (for all, see Synonyms field below)
Comment
Links to External Ontologies
DO.org
Annotations
Records annotated with this term OR any of its CHILD TERMS
    Results list data from multiple species. Click on a button above and use the 'Filter by species' options on the resulting HitList to retrieve species-specific data.
Records annotated with this exact term (annotations to child terms are NOT included)
No relevant records available
show Full annotation statements including this term (annotations to child terms are NOT included), and relevant FlyBase records
 Full annotation statements 
Relevant FlyBase reports
 Genes
 mucopolysaccharidosis VI       2
 for disease ribbon | mucopolysaccharidosis VI       2
 model of | mucopolysaccharidosis VI       2
Spanning Tree (Parents/Children)
Only view relationship: 
  lysosomal storage disease
   |__mucopolysaccharidosis
       |__mucopolysaccharidosis VI  2 rec.
Spanning Tree View Settings
Parents/Children
View Depth
Show hierarchy levels: for parents, for children
hide Relationships
Is a mucopolysaccharidosis
Part of
hide Synonyms & Secondary IDs
Synonyms
  • "arylsulfatase B deficiency" EXACT
    "deficiency of N-acetylgalactosamine-4-sulfatase" EXACT
    "Maroteaux - Lamy syndrome" EXACT
    "Maroteaux-Lamy syndrome" EXACT
    "MPS VI - Maroteaux-Lamy syndrome" EXACT
Secondary IDs
hide External Crossreferences & Linkouts
GARD:7095
MESH:D009087
MIM:253200
NCI:C61264
SNOMEDCT_US_2023_03_01:69463008
UMLS_CUI:C0026709