uroporphyrinogen

(redirected from uroporphyrinogen I synthetase)

uroporphyrinogen

 [u″ro-por″fĭ-rin´o-jen]
a porphyrinogen formed from porphobilinogen; it is a precursor of uroporphyrin and coproporphyrinogen.

por·phy·rin·o·gens

(pōr'fi-rin'ō-jenz),
Intermediates in the biosynthesis of heme, as follows: four porphobilinogens condense to form uroporphyrinogens I and III (giving rise to side products uroporphyrins I and III) that are decarboxylated to form coproporphyrinogens I and III (giving rise to side products coproporphyrins I and III); coproporphyrinogen III is oxidized to protoporphyrinogen III (IX), which is then oxidized to form protoporphyrin III (IX) (this last intermediate adds ferrous iron to yield heme); certain porphyrinogens are elevated in certain porphyrias.

por·phy·rin·o·gens

(pōr'fir-in'ō-jenz)
Intermediates in the biosynthesis of heme; certain porphyrinogens are elevated in certain porphyrias.
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References in periodicals archive ?
This is due to mutations in the gene encoding for uroporphyrinogen I synthetase. (3), (4) In our case, diagnosis was made after the recognition of dark urine and higher than normal urine porphobilinogen, corpoporphyrin and total porphyrin levels.
Coexistence of familial Mediterranean fever and acute intermittent porphyria / Gebe bir hastada ailesel Akdeniz atesi ve akut intermittan porfiri birlikteligi
Decreased red cell uroporphyrinogen I synthetase activity in intermittent acute porphyria.
Variations in erythrocyte uroporphyrinogen I synthetase activity in non porphyrias.
Molecular and biochemical studies of acute intermittent porphyria in 196 patients and their families

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