鉄付加酵素

鉄付加酵素（てつふかこうそ、ferrochelatase (FECH)）は、プロトポルフィリンIXをヘムに変換するヘム生合成8段階の最後の反応酵素である。プロトポルフィリンにFe2+を付加する反応を触媒する。触媒反応は、 プロトポルフィリン + Fe++ ↔ プロトヘム + 2 H+ 鉄付加酵素は、497個のアミノ残基で構成され、分子量は55.4 kDaである。 + Fe2+ ⇔ + 2H+ プロトポルフィリンIX ヘム 鉄付加酵素は、ミトコンドリアに所在し、ヘム生合成においてプロトポルフィリンIXに鉄（Fe2+）を付加する反応を触媒する。鉄付加酵素の欠乏は、骨髄性プロトポルフィリアの発症に関係する。この遺伝について、異なる異性体を記述した2つの転写変異が見つかっている。 ^ “Entrez Gene: FECH ferrochelatase (protoporphyria)”. 2010年7月10日閲覧。 ^ a b c GRCh38: Ensembl release 89: ENSG00000066926 - Ensembl, May 2017 ^ a b c GRCm38: Ensembl release 89: ENSMUSG00000024588 - Ensembl, May 2017 ^ Human PubMed Reference: ^ Mouse PubMed Reference: ^ Taketani, Shigeru; Kakimoto Kazuhiro, Ueta Hiromi, Masaki Ryuichi, Furukawa Takako (Apr. 2003). “Involvement of ABC7 in the biosynthesis of heme in erythroid cells: interaction of ABC7 with ferrochelatase”. Blood (United States) 101 (8): 3274–80. doi:10.1182/blood-2002-04-1212. ISSN 0006-4971. PMID 12480705.  Cox TM (1997). “Erythropoietic protoporphyria.”. J. Inherit. Metab. 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