Ferrochelatase

Ferrochelatase (FECH, protoheme ferrolyase) is an enzyme that catalyses the terminal (eighth) step in the biosynthesis of heme, converting protoporphyrin IX into heme. It catalyses reaction: protoporphyrin + Fe⁺⁺ ↔ protoheme + 2 H⁺. A ferrochelatase enzyme consists of 497 amino acid residues with a m.w. of 55.4 kDa.cite web | title = Entrez Gene: FECH ferrochelatase (protoporphyria)| url = http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=2235| accessdate = ]

PBB_Summary
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summary_text = Ferrochelatase is localized to the mitochondrion where it catalyzes the insertion of the ferrous form of iron into protoporphyrin IX in the heme synthesis pathway. Defects in ferrochelatase are associated with erythropoietic protoporphyria. Two transcript variants encoding different isoforms have been found for this gene.cite web | title = Entrez Gene: FECH ferrochelatase (protoporphyria)| url = http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=2235| accessdate = ]

ee also

* Lyases
* Erythropoietic protoporphyria
* Sirohydrochlorin ferrochelatase

References

Further reading

PBB_Further_reading
citations =
*cite journal | author=Cox TM |title=Erythropoietic protoporphyria. |journal=J. Inherit. Metab. Dis. |volume=20 |issue= 2 |pages= 258–69 |year= 1997 |pmid= 9211198 |doi=
*cite journal | author=Buller RE, Schrader WT, O'Maller BW |title=Steroids and the practical aspects of performing binding studies. |journal=J. Steroid Biochem. |volume=7 |issue= 5 |pages= 321–6 |year= 1976 |pmid= 180343 |doi=
*cite journal | author=Bonkowsky HL, Bloomer JR, Ebert PS, Mahoney MJ |title=Heme synthetase deficiency in human protoporphyria. Demonstration of the defect in liver and cultured skin fibroblasts. |journal=J. Clin. Invest. |volume=56 |issue= 5 |pages= 1139–48 |year= 1976 |pmid= 1184741 |doi=
*cite journal | author=Brenner DA, Didier JM, Frasier F, "et al." |title=A molecular defect in human protoporphyria. |journal=Am. J. Hum. Genet. |volume=50 |issue= 6 |pages= 1203–10 |year= 1992 |pmid= 1376018 |doi=
*cite journal | author=Nakahashi Y, Fujita H, Taketani S, "et al." |title=The molecular defect of ferrochelatase in a patient with erythropoietic protoporphyria. |journal=Proc. Natl. Acad. Sci. U.S.A. |volume=89 |issue= 1 |pages= 281–5 |year= 1992 |pmid= 1729699 |doi=
*cite journal | author=Lamoril J, Boulechfar S, de Verneuil H, "et al." |title=Human erythropoietic protoporphyria: two point mutations in the ferrochelatase gene. |journal=Biochem. Biophys. Res. Commun. |volume=181 |issue= 2 |pages= 594–9 |year= 1992 |pmid= 1755842 |doi=
*cite journal | author=Diep A, Li C, Klisak I, "et al." |title=Assignment of the gene for cyclic AMP-response element binding protein 2 (CREB2) to human chromosome 2q24.1-q32. |journal=Genomics |volume=11 |issue= 4 |pages= 1161–3 |year= 1992 |pmid= 1838349 |doi=
*cite journal | author=Nakahashi Y, Taketani S, Okuda M, "et al." |title=Molecular cloning and sequence analysis of cDNA encoding human ferrochelatase. |journal=Biochem. Biophys. Res. Commun. |volume=173 |issue= 2 |pages= 748–55 |year= 1991 |pmid= 2260980 |doi=
*cite journal | author=Rossi E, Attwood PV, Garcia-Webb P, Costin KA |title=Inhibition of human lymphocyte ferrochelatase activity by hemin. |journal=Biochim. Biophys. Acta |volume=1038 |issue= 3 |pages= 375–81 |year= 1990 |pmid= 2340297 |doi=
*cite journal | author=Polson RJ, Lim CK, Rolles K, "et al." |title=The effect of liver transplantation in a 13-year-old boy with erythropoietic protoporphyria. |journal=Transplantation |volume=46 |issue= 3 |pages= 386–9 |year= 1988 |pmid= 3047929 |doi=
*cite journal | author=Bonkovsky HL, Schned AR |title=Fatal liver failure in protoporphyria. Synergism between ethanol excess and the genetic defect. |journal=Gastroenterology |volume=90 |issue= 1 |pages= 191–201 |year= 1986 |pmid= 3940245 |doi=
*cite journal | author=Prasad AR, Dailey HA |title=Effect of cellular location on the function of ferrochelatase. |journal=J. Biol. Chem. |volume=270 |issue= 31 |pages= 18198–200 |year= 1995 |pmid= 7629135 |doi=
*cite journal | author=Sarkany RP, Alexander GJ, Cox TM |title=Recessive inheritance of erythropoietic protoporphyria with liver failure. |journal=Lancet |volume=343 |issue= 8910 |pages= 1394–6 |year= 1994 |pmid= 7910885 |doi=
*cite journal | author=Tugores A, Magness ST, Brenner DA |title=A single promoter directs both housekeeping and erythroid preferential expression of the human ferrochelatase gene. |journal=J. Biol. Chem. |volume=269 |issue= 49 |pages= 30789–97 |year= 1995 |pmid= 7983009 |doi=
*cite journal | author=Maruyama K, Sugano S |title=Oligo-capping: a simple method to replace the cap structure of eukaryotic mRNAs with oligoribonucleotides. |journal=Gene |volume=138 |issue= 1-2 |pages= 171–4 |year= 1994 |pmid= 8125298 |doi=
*cite journal | author=Dailey HA, Sellers VM, Dailey TA |title=Mammalian ferrochelatase. Expression and characterization of normal and two human protoporphyric ferrochelatases. |journal=J. Biol. Chem. |volume=269 |issue= 1 |pages= 390–5 |year= 1994 |pmid= 8276824 |doi=
*cite journal | author=Wang X, Poh-Fitzpatrick M, Carriero D, "et al." |title=A novel mutation in erythropoietic protoporphyria: an aberrant ferrochelatase mRNA caused by exon skipping during RNA splicing. |journal=Biochim. Biophys. Acta |volume=1181 |issue= 2 |pages= 198–200 |year= 1993 |pmid= 8481408 |doi=
*cite journal | author=Nakahashi Y, Miyazaki H, Kadota Y, "et al." |title=Molecular defect in human erythropoietic protoporphyria with fatal liver failure. |journal=Hum. Genet. |volume=91 |issue= 4 |pages= 303–6 |year= 1993 |pmid= 8500787 |doi=
*cite journal | author=Imoto S, Tanizawa Y, Sato Y, "et al." |title=A novel mutation in the ferrochelatase gene associated with erythropoietic protoporphyria. |journal=Br. J. Haematol. |volume=94 |issue= 1 |pages= 191–7 |year= 1996 |pmid= 8757534 |doi=
*cite journal | author=Crouse BR, Sellers VM, Finnegan MG, "et al." |title=Site-directed mutagenesis and spectroscopic characterization of human ferrochelatase: identification of residues coordinating the [2Fe-2S] cluster. |journal=Biochemistry |volume=35 |issue= 50 |pages= 16222–9 |year= 1997 |pmid= 8973195 |doi= 10.1021/bi9620114

External links

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