FECH Catalyzes the ferrous insertion into protoporphyrin IX. Defects in FECH are the cause of erythropoietic protoporphyria (EPP). Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver. EPP is a form of porphyria marked by excessive protoporphyrin in erythrocytes, plasma, liver and feces, and by widely varying photosensitive skin changes ranging from a burning or pruritic sensation to erythema, edema and wheals. Belongs to the ferrochelatase family. 2 isoforms of the human protein are produced by alternative splicing. Note: This description may include information from UniProtKB.
Protein type: Cofactor and Vitamin Metabolism - porphyrin and chlorophyll; EC 4.99.1.1; Lyase; Mitochondrial
Chromosomal Location of Human Ortholog: 18 E1|18 37.43 cM
Cellular Component:  membrane; mitochondrial inner membrane; mitochondrion
Molecular Function:  2 iron, 2 sulfur cluster binding; ferrochelatase activity; heme binding; iron ion binding; iron-responsive element binding; iron-sulfur cluster binding; lyase activity; metal ion binding; protein binding; tetrapyrrole binding
Biological Process:  cholesterol metabolic process; detection of UV; erythrocyte differentiation; heme biosynthetic process; iron ion homeostasis; porphyrin-containing compound biosynthetic process; protoporphyrinogen IX metabolic process; regulation of eIF2 alpha phosphorylation by heme; regulation of gene expression; regulation of hemoglobin biosynthetic process; response to light stimulus; very-low-density lipoprotein particle assembly
Reference #:  P22315 (UniProtKB)
Alt. Names/Synonyms: AI894116; fch; Fcl; Fech; ferrochelatase; Ferrochelatase, mitochondrial; Heme synthase; heme synthetase; HEMH; Protoheme ferro-lyase
Gene Symbols: Fech
Molecular weight: 47,130 Da
Basal Isoelectric point: 9.1  Predict pI for various phosphorylation states
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FECH

Protein Structure Not Found.


Cross-references to other databases:  STRING  |  Reactome  |  BioGPS  |  Pfam  |  ENZYME  |  Phospho.ELM  |  NetworKIN  |  UniProtKB  |  Entrez-Gene  |  Ensembl Gene