FB2025_05 , released December 11, 2025
FB2025_05 , released December 11, 2025
CV Term Report
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General Information
Term 3-methylglutaconic aciduria type 1 ID (Ontology) DOID:0110002 (Human Disease)
Definition A 3-methylglutaconic aciduria that has_material_basis_in homozygous or compound heterozygous mutation in the AUH gene on chromosome 9q22.
Also Known As "3-methylglutaconic aciduria type I" ; "3-methylglutaconyl-CoA hydratase deficiency" ; "3MG-CoA hydratase deficiency" (for all, see Synonyms field below)
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Data Class Field Records
Alleles (FBal)  HUMAN_DISEASE_INTERACTIONS       1
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 Alleles Genes
 3-methylglutaconic aciduria type 1       1      2
 for disease ribbon | 3-methylglutaconic aciduria type 1       --       2
 model of | 3-methylglutaconic aciduria type 1       1      2
Spanning Tree (Parents/Children)
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autosomal genetic disease
 |__autosomal recessive disease__
organic acidemia                 |
 |__3-methylglutaconic aciduria__|
                                 3-methylglutaconic aciduria type 1  3 rec.
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Is a autosomal recessive disease
3-methylglutaconic aciduria
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Synonyms
  • "3-methylglutaconic aciduria type I" EXACT
    "3-methylglutaconyl-CoA hydratase deficiency" EXACT
    "3MG-CoA hydratase deficiency" EXACT
    "MGA type I" EXACT
    "MGA1" EXACT OMO:0003012
Secondary IDs
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MESH:C562801
MIM:250950
ORDO:67046