DISEASES

Disease-gene associations mined from literature

Human genes for dihydropyrimidinase deficiency

Dihydropyrimidinase deficiency [DOID:0111629]

A pyrimidine metabolic disorder characterized by a defect in the degredation of uracil and thymine resulting in elevated levels of 5,6-dihydrouracil and 5,6-dihydrothymine in urine that has_material_basis_in homozygous or compound heterozygous mutation in DPYS on chromosome 8q22.3.

Synonyms:  dihydropyrimidinase deficiency,  dihydropyrimidinase deficiencies,  DOID:0111629,  dihydropyrimidinuria,  DPH deficiency ...