Ferrochelatase

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Ferrochelatase
Identifiers
Symbol Ferrochelatase
Pfam PF00762
InterPro IPR001015
PROSITE PDOC00462
SCOP 1ak1
SUPERFAMILY 1ak1
OPM superfamily 137
OPM protein 1hrk
Ferrochelatase
Protein FECH PDB 1hrk.png
PDB rendering based on 1hrk.
Available structures
PDB Ortholog search: PDBe, RCSB
Identifiers
Symbols FECH ; EPP; FCE
External IDs OMIM612386 MGI95513 HomoloGene113 GeneCards: FECH Gene
EC number 4.99.1.1
RNA expression pattern
PBB GE FECH 203116 s at tn.png
PBB GE FECH 203115 at tn.png
More reference expression data
Orthologs
Species Human Mouse
Entrez 2235 14151
Ensembl ENSG00000066926 ENSMUSG00000024588
UniProt P22830 P22315
RefSeq (mRNA) NM_000140 NM_007998
RefSeq (protein) NP_000131 NP_032024
Location (UCSC) Chr 18:
55.22 – 55.25 Mb
Chr 18:
64.46 – 64.49 Mb
PubMed search [1] [2]

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 the reaction:

protoporphyrin + Fe++protoheme + 2 H+.

A ferrochelatase enzyme consists of 497 amino acid residues with a m.w. of 55.4 kDa.[1]

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.[1]

Heme synthesis—note that some reactions occur in the cytoplasm and some in the mitochondrion (yellow)

Interactions[edit]

Ferrochelatase has been shown to interact with ABCB7.[2]

See also[edit]

References[edit]

  1. ^ a b "Entrez Gene: FECH ferrochelatase (protoporphyria)". 
  2. ^ Taketani, Shigeru; Kakimoto Kazuhiro; Ueta Hiromi; Masaki Ryuichi; Furukawa Takako (Apr 2003). "Involvement of ABC7 in the biosynthesis of heme in erythroid cells: interaction of ABC7 with ferrochelatase". Blood (United States) 101 (8): 3274–80. doi:10.1182/blood-2002-04-1212. ISSN 0006-4971. PMID 12480705. 

Further reading[edit]

External links[edit]