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ferrochelatase, mitochondrial; heme synthase; protoheme ferro-lyase (FECH)
Function:
- catalyzes the ferrous insertion into protoporphyrin IX
- porphyrin metabolism; protoheme biosynthesis; protoheme from protoporphyrin-IX: step 1/1
Cofactor: binds 1 2Fe-2S cluster
Inhibition:
- inhibited by nitric oxide (NO)
- the 2Fe-2S cluster could act as a NO sensor
protoheme + 2 H+ = protoporphyrin + Fe+2
Structure:
- homodimer
- belongs to the ferrochelatase family
Compartment:
- mitochondrial inner membrane; matrix side
Pathology:
- defects in FECH are the cause of erythropoietic protoporphyria
Interactions
molecular events
Related
FECH gene mutation
General
lyase
mitochondrial protein
Properties
SIZE: entity length = 423 aa
MW = 48 kD
COMPARTMENT: mitochondrial matrix
MOTIF: cofactor-binding site [196-196]
COFACTOR-BOUND: iron sulfur [FeS] cluster
histidine residue {H230}
aspartate residue {D383}
cofactor-binding site [403-403]
COFACTOR-BOUND: iron sulfur [FeS] cluster
cofactor-binding site [406-406]
COFACTOR-BOUND: iron sulfur [FeS] cluster
cofactor-binding site [411-411]
COFACTOR-BOUND: iron sulfur [FeS] cluster
MISC-INFO: INHIBITOR = PB+2
Database Correlations
OMIM correlations
MORBIDMAP 612386
UniProt P22830
Pfam PF00762
Entrez Gene 2235
Kegg hsa:2235
ENZYME 4.99.1.1
References
- UniProt :accession P22830
- GeneReviews
http://www.ncbi.nlm.nih.gov/sites/genetests/lab/gene/FECH
- Wikipedia; Note: ferrochelatase entry
http://en.wikipedia.org/wiki/ferrochelatase