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Publication : Biochemistry of Mitochondrial Coenzyme Q Biosynthesis.

First Author  Stefely JA Year  2017
Journal  Trends Biochem Sci Volume  42
Issue  10 Pages  824-843
PubMed ID  28927698 Mgi Jnum  J:320153
Mgi Id  MGI:6869992 Doi  10.1016/j.tibs.2017.06.008
Citation  Stefely JA, et al. (2017) Biochemistry of Mitochondrial Coenzyme Q Biosynthesis. Trends Biochem Sci 42(10):824-843
abstractText  Coenzyme Q (CoQ, ubiquinone) is a redox-active lipid produced across all domains of life that functions in electron transport and oxidative phosphorylation and whose deficiency causes human diseases. Yet, CoQ biosynthesis has not been fully defined in any organism. Several proteins with unclear molecular functions facilitate CoQ biosynthesis through unknown means, and multiple steps in the pathway are catalyzed by currently unidentified enzymes. Here we highlight recent progress toward filling these knowledge gaps through both traditional biochemistry and cutting-edge 'omics' approaches. To help fill the remaining gaps, we present questions framed by the recently discovered CoQ biosynthetic complex and by putative biophysical barriers. Mapping CoQ biosynthesis, metabolism, and transport pathways has great potential to enhance treatment of numerous human diseases.
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