4-hydroxyphenylpyruvate dioxygenase


  • Accession: P32754
  • Swissprot: HPPD_HUMAN
  • Organism: Homo sapiens
  • Gene: HPD
  • Target class: Enzyme

Drug Relations:

Nitisinone is a competitive inhibitor of 4-hydroxyphenyl-pyruvate dioxygenase, an enzyme upstream of FAH in the tyrosine catabolic pathway. By inhibiting the normal catabolism of tyrosine in patients with hereditary tyrosinemia type 1 (HT-1), nitisinone prevents the accumulation of the catabolic intermediates maleylacetoacetate and fumarylacetoacetate. In patients with HT-1, these catabolic intermediates are converted to the toxic metabolites succinylacetone and succinylacetoacetate, which are responsible for the observed liver and kidney toxicity. Bioactivity details MOA