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

More general terms

• lyase
• mitochondrial protein

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=2235
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:2235
• OMIM: https://mirror.omim.org/entry/177000
• OMIM: https://mirror.omim.org/entry/612386
• UniProt: http://www.uniprot.org/uniprot/P22830.html