Names & Taxonomy
- Uniprot ID:
- P22830
- Entry Name:
- HEMH_HUMAN
- Status:
- reviewed
- Protein Names:
- Ferrochelatase, mitochondrial (EC 4.99.1.1) (Heme synthase) (Protoheme ferro-lyase)
- Gene Names:
- FECH
- Gene Names Primary:
- FECH
- Organism:
- Homo sapiens (Human)
Structure
- Length:
- 423
- Sequence:
- MRSLGANMAAALRAAGVLLRDPLASSSWRVCQPWRWKSGAAAAAVTTETAQHAQGAKPQVQPQKRKPKTGILMLNMGGPETLGDVHDFLLRLFLDRDLMTLPIQNKLAPFIAKRRTPKIQEQYRRIGGGSPIKIWTSKQGEGMVKLLDELSPNTAPHKYYIGFRYVHPLTEEAIEEMERDGLERAIAFTQYPQYSCSTTGSSLNAIYRYYNQVGRKPTMKWSTIDRWPTHHLLIQCFADHILKELDHFPLEKRSEVVILFSAHSLPMSVVNRGDPYPQEVSATVQKVMERLEYCNPYRLVWQSKVGPMPWLGPQTDESIKGLCERGRKNILLVPIAFTSDHIETLYELDIEYSQVLAKECGVENIRRAESLNGNPLFSKALADLVHSHIQSNELCSKQLTLSCPLCVNPVCRETKSFFTSQQL
- Proteomes:
- UP000005640
Subcellular location
- Subcellular Location:
- Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.
Function
- Function:
- Catalyzes the ferrous insertion into protoporphyrin IX.
- Pathway:
- Porphyrin-containing compound metabolism; protoheme biosynthesis; protoheme from protoporphyrin-IX: step 1/1.
- Catalytic Activity:
- Protoheme + 2 H(+) = protoporphyrin + Fe(2+).
- Cofactor:
- COFACTOR: Name= cluster; Xref=ChEBI:CHEBI:49601; ; Note=Binds 1 cluster.;
- Enzyme Regulation:
- ENZYME REGULATION: Inhibited by nitric oxide (NO). The 2Fe-2S cluster could act as a NO sensor.
- Active Site:
- ACT_SITE 230 230; ACT_SITE 383 383
- Cross Reference Drug Bank:
- DB02659
- Gene Ontology Go:
- mitochondrial inner membrane
mitochondrial matrix
mitochondrion
2 iron, 2 sulfur cluster binding
ferrochelatase activity
ferrous iron binding
cellular response to dexamethasone stimulus
generation of precursor metabolites and energy
heme biosynthetic process
porphyrin-containing compound metabolic process
protoporphyrinogen IX metabolic process
response to arsenic-containing substance
response to drug
response to ethanol
response to insecticide
response to lead ion
response to light stimulus
response to methylmercury
response to platinum ion
small molecule metabolic process - Gene Ontology Biological Process:
- cellular response to dexamethasone stimulus
generation of precursor metabolites and energy
heme biosynthetic process
porphyrin-containing compound metabolic process
protoporphyrinogen IX metabolic process
response to arsenic-containing substance
response to drug
response to ethanol
response to insecticide
response to lead ion
response to light stimulus
response to methylmercury
response to platinum ion
small molecule metabolic process - Gene Ontology Molecular Function:
- 2 iron, 2 sulfur cluster binding
ferrochelatase activity
ferrous iron binding - Gene Ontology Cellular Component:
- mitochondrial inner membrane
mitochondrial matrix
mitochondrion - Keywords:
- 2Fe-2S
3D-structure
Acetylation
Alternative splicing
Complete proteome
Disease mutation
Heme biosynthesis
Iron
Iron-sulfur
Lyase
Membrane
Metal-binding
Mitochondrion
Mitochondrion inner membrane
Polymorphism
Porphyrin biosynthesis
Reference proteome
Transit peptide