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The human mitochondrial enzyme BCO2 exhibits catalytic activity towards carotenoids and apocarotenoids

J Biol Chem. 2020; 
Linda D Thomas, Sepalika Bandara, Vipul M Parmar, Ramkumar Srinivasagan, Nimesh Khadka, Marcin Golczak, Philip D Kiser, Johannes von Lintig
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Molecular Biology Reagents … Synthetic codon usage optimized human BCO2 variants, 522-aa and 519-aa, were custom made by GenScript (Piscataway, NJ) … A chimeric mouse BCO2 with the four amino acid insertion, GKAA, was custom made by GenScript (Piscataway, NJ) … Get A Quote

摘要

The enzyme β-carotene-oxygenase 2 (BCO2) converts carotenoids into more polar metabolites. Studies in mammals, fish and birds revealed that BCO2 controls carotenoid homeostasis and is involved in the pathway for vitamin A production. However, it is controversial whether BCO2 function is conserved in humans, owing to a four amino acid long insertion caused by a splice acceptor site polymorphism. We here show that human BCO2 splice variants, BCO2a and BCO2b, are expressed as pre-proteins with mitochondrial targeting sequence (MTS). The MTS of BCO2a directed a green fluorescent reporter protein to the mitochondria when expressed in ARPE-19 cells. Removal of the MTS increased solubility of BCO2a when expressed in ... More

关键词

BCO2, apo-carotenoid, carotenoid, dioxygenase, macular pigments, metabolism, retina, vision