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FLASH GENE
Symbol FECH contributors: mct/npt/pgu - updated : 21-03-2019
HGNC name ferrochelatase (protoporphyria)
HGNC id 3647
EXPRESSION
Type ubiquitous
   expressed in (based on citations)
organ(s)
SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
Cardiovascularheart   highly
Digestiveliver   highly
Reproductivefemale systemuteruscervix highly
cell lineage
cell lines
fluid/secretion
at STAGE
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • a 62 AAs leader sequence is thought to target FECH to mitochondria where it is cleaved to produce the active mature protein
  • mono polymer homomer , dimer
    isoforms Precursor synthesised on cytosolic ribosomes as a preprotein with a cleavable presequence at its amino-terminus
    HOMOLOGY
    interspecies homolog to murine Fech
    Homologene
    FAMILY
  • ferrochelatase family
  • CATEGORY enzyme , storage
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria,inner
    intracellular,cytoplasm,organelle,mitochondria,matrix
    text localizes to both the mitochondrial outer and inner membranes and that the change in the equilibrium position of the forward and reverse activities may be regulated by the phosphorylation of ferrochelatase
    basic FUNCTION
  • catalyzing heme formation
  • catalyzes the terminal step in the heme biosynthetic pathway, the insertion of ferrous iron into protoporphyrin IX
  • also participates in the reverse reaction of iron removal from heme
  • terminal enzyme in heme biosynthesis and catalyzes the insertion of ferrous iron into protoporphyrin IX to form protoheme IX (heme)
  • catalyzes the insertion of ferrous iron into protoporphyrin IX to form heme
  • ferrochelatase (FECH), inserts iron into protoporphyrin IX (PPIX) to form heme
  • PGRMC1 may be likely a heme chaperone or sensor
  • FECH is an enzyme necessary for heme synthesis, which is essential for maintaining normal functions of endothelial nitric oxide synthase (eNOS) and soluble guanylyl cyclase (sGC)
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism porphyrin/heme
    signaling
    protoheme biosynthesis
    a component
  • homodimeric
  • FECH forms an oligomeric complex with SLC25A37 and ABCB10 to synergistically integrate mitochondrial iron importation and use for heme biosynthesis
  • a dimeric FECH physically bridged ABCB7 and ABCB10 homodimers by binding near the NBDs of each ABC transporter
  • INTERACTION
    DNA
    RNA
    small molecule cofactor,
  • binds 1 2Fe-2S cluster
  • protein
  • SLC25A37 is a mitochondrial transporter of Fe used for heme formation by FECH and for 2Fe2S cluster synthesis, which is critical to FECH activity/stability
  • ATPIF1 regulates the catalytic efficiency of vertebrate FECH to synthesize haem
  • PGRMC1 may regulate FECH activity by controlling heme release
  • cell & other
    REGULATION
    inhibited by by nitric oxide (NO)
    ASSOCIATED DISORDERS
    corresponding disease(s) EPP
    related resource MITOP database
    Susceptibility to erythropoietic protoporphyria
    Variant & Polymorphism SNP , other
  • modulating the use of a constitutive aberrant acceptor splice site
  • IVS3-48C allele increasing the risk of erythropoietic protoporphyria
  • Candidate gene
    Marker
    Therapy target
    SystemTypeDisorderPubmed
    neurosensorialvisualdegenerative
    FECH inhibition could be used therapeutically to block ocular neovascularization in DMLA
    ANIMAL & CELL MODELS