encephalopathy due to urocanase deficiencyurocanic aciduriaUROCDurocanate hydratase deficiencyhigh urine urocanic acid levels
obo:hasOBONamespace
disease_ontology
n2:IAO_0000115
A histidine metabolism disease characterized by urocanic aciduria and other variable manifestations including intellectual disability and intermittent ataxia that has_material_basis_in mutation homozygous or compound heterozygous in UROC1 on chromosome 3q21.3.