ferrochelatase


Also found in: Acronyms, Encyclopedia, Wikipedia.

fer·ro·che·la·tase

(fer'ō-kē'lă-tās),
A lyase that catalyzes the reversible acid hydrolysis of heme, forming protoporphyrin IX and free ferrous iron; inhibited by lead; a deficiency of ferrochelatase results in erythropoietic protoporphyria.

ferrochelatase

A MITOCHONDRIAL enzyme that catalyzes the process by which iron is incorporated into the PROTOPORPHYRIN molecule in the process of forming HAEMOGLOBIN.

ferrochelatase

an enzyme involved in the incorporation of elemental iron into the hemoglobin synthesis cycle. Called also heme synthetase.
References in periodicals archive ?
In contrast, the weaker binding of lead to ALAD1 results in more bioavailable lead that can inhibit ferrochelatase.
As mentioned above, the two target sites in the biosynthetic pathway of heine by PbB are the sites of activity of ALAD and ferrochelatase (Moore and Goldberg 1985).
Ferrochelatase activity in the proband was reduced to 25% of the mean value, as is typical for EPP patients (5).
Direct sequencing of the PCR-amplified fragment containing exon 5 revealed a missense mutation, T545G, which led to the conversion of Leu-182 to Arg in ferrochelatase.
However, subsequent research on ferrochelatase revealed that this enzyme catalyzes zinc as well as iron chelation by protoporphyrin (12,13).
An important exception to these ratios occurs with protoporphyria, in which an inherited ferrochelatase deficiency leads to a massive overproduction and accumulation of EP (11).
This porphyria is induced by the inhibition of ferrochelatase probably caused by a griseofulvin adduct of protoporphyrin IX produced in a cytochrome P450 (Cyp)-mediated suicide reaction (Bellingham et al.
Coproporphyrinogen oxidase, protoporphyrinogen oxidase, and ferrochelatase activities were assayed using lymphocytes, according to the methods reported previously (13-15).
Modulation of the phenotype in dominant erythropoietic protoporphyria by a low expression of the normal ferrochelatase allele.