作者: Isabel González-Mariscal , Elena García-Testón , Sergio Padilla , Alejandro Martín-Montalvo , Teresa Pomares Viciana
DOI: 10.1159/000362897
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摘要: Coenzyme Q (CoQ) is a mitochondrial lipid, which functions mainly as an electron carrier from complex I or II to III at the inner membrane, and also antioxidant in cell membranes. CoQ needed acceptor β-oxidation of fatty acids pyridine nucleotide biosynthesis, it responsible for opening permeability transition pore. The yeast model has been very useful analyze synthesis CoQ, therefore, most knowledge about its regulation was obtained Saccharomyces cerevisiae model. biosynthesis regulated support 2 processes: bioenergetic metabolism defense. Alterations carbon source yeast, nutrient availability yeasts mammalian cells, upregulate genes encoding proteins involved synthesis. Oxidative stress, generated by chemical physical agents serum deprivation, modifies specifically expression some COQ means stress transcription factors such Msn2/4p, Yap1p Hsf1p. In general, induction gene produced metabolic changes modulated downstream other regulatory mechanisms protein import mitochondria, assembly multi-enzymatic composed Coq existence phosphorylation cycle that regulates last steps biosynthesis. biosynthetic starts with production nucleating lipid HHB action Coq2 protein. Then, Coq4 recognizes precursor acting nucleus complex. activity Coq8p, probably kinase, allows formation initial pre-complex containing all exception Coq7p. This leads 5-demethoxy-Q6 (DMQ6), Coq7p substrate. When de novo required, becomes dephosphorylated Ptc7p increasing rate CoQ6. critical better understanding Taking into account patients CoQ10 deficiency maintain extent machinery synthesize new promising strategies treatment will require future.