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4- hydroxybenzoate polyprenyltransferase, mitochondrial Ubiquinone biosynthesis O- methyltransferase, mitochondrial Ubiquinone biosynthesis monooxygenase COQ6, mitochondrial 2-methoxy-6- polyprenyl- 1,4- benzoquinol methylase, mitochondrial 5- demethoxyubiquinone hydroxylase, mitochondrial Ubiquinone biosynthesis O- methyltransferase, mitochondrial 4- Hydroxybenzoic acid All-trans-hexaprenyl diphosphate 3-Hexaprenyl-4-hydroxybenzoic acid PPi 3-Hexaprenyl-4,5-Dihydroxybenzoic acid S-Adenosylmethionine S-Adenosylhomocysteine 3-Hexaprenyl-4-hydroxy-5-methoxybenzoic acid 2-Hexaprenyl-6-methoxyphenol O2 2-Hexaprenyl-6-methoxy-1,4-benzoquinone H2O S-Adenosylmethionine 2-Hexaprenyl-3-methyl-6-methoxy-1,4 benzoquinone S-Adenosylhomocysteine O2 NADPH H+ 2-Hexaprenyl-3-methyl-5-hydroxy-6-methoxy- 1,4-benzoquinone NADP H2O S-Adenosylmethionine Ubiquinone-6 S-Adenosylhomocysteine Fe3+ Cytosol Mitochondrion Mitochondrial Membrane
Mitochondria coq-2 coq-3 coq-6 coq-5 clk-1 coq-3 4- Hydroxybenzoic acid All-trans- hexaprenyl diphosphate 3-Hexaprenyl- 4- hydroxybenzoic acid Pyrophosphate 3-Hexaprenyl- 4,5- Dihydroxybenzoic acid S- Adenosylmethionine S- Adenosylhomocysteine 3-Hexaprenyl- 4-hydroxy-5- methoxybenzoic acid 2-Hexaprenyl- 6- methoxyphenol Oxygen 2-Hexaprenyl- 6-methoxy- 1,4- benzoquinone Water S- Adenosylmethionine 2-Hexaprenyl- 3-methyl-6- methoxy-1,4 benzoquinone S- Adenosylhomocysteine Oxygen NADPH Hydrogen Ion 2-Hexaprenyl- 3-methyl-5- hydroxy-6- methoxy-1,4- benzoquinone NADP Water S- Adenosylmethionine Ubiquinone-6 S- Adenosylhomocysteine
coq-2 coq-3 coq-6 coq-5 clk-1 coq-3 4-HBA ATHD 3H4H PPi Dhhpba SAMe SAH 3-MHHB 2h6m O2 2H6M14B H2O SAMe hmm14b SAH O2 NADPH H+ hmhm14B NADP H2O SAMe Ubiq6 SAH Fe3+ Cytosol Mitochondrion Mitochondrial Membrane
Mitochondria coq-2 coq-3 coq-6 coq-5 clk-1 coq-3 4-HBA ATHD 3H4H Ppi Dhhpba SAMe SAH 3-MHHB 2h6m O2 2H6M14B H2O SAMe hmm14b SAH O2 NADPH H+ hmhm14B NADP H2O SAMe Ubiq6 SAH