Page last updated: 2024-10-06

Riboflavin and CoQ disorders

Proteins (14)

ProteinSynonymsTaxonomy
AprataxinEC 3.6.1.71; EC 3.6.1.72; Forkhead-associated domain histidine triad-like protein; FHA-HITHomo sapiens (human)
Electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrialETF-QO; ETF-ubiquinone oxidoreductase; EC 1.5.5.1; Electron-transferring-flavoprotein dehydrogenase; ETF dehydrogenaseHomo sapiens (human)
5-demethoxyubiquinone hydroxylase, mitochondrialDMQ hydroxylase; EC 1.14.99.60; Timing protein clk-1 homolog; Ubiquinone biosynthesis monooxygenase COQ7Homo sapiens (human)
Atypical kinase COQ8A, mitochondrialEC 2.7.-.-; Chaperone activity of bc1 complex-like; Chaperone-ABC1-like; Coenzyme Q protein 8A; aarF domain-containing protein kinase 3Homo sapiens (human)
FAD synthaseEC 2.7.7.2; FAD pyrophosphorylase; FMN adenylyltransferase; Flavin adenine dinucleotide synthaseHomo sapiens (human)
Riboflavin kinaseEC 2.7.1.26; ATP:riboflavin 5'-phosphotransferase; FlavokinaseHomo sapiens (human)
Ubiquinone biosynthesis protein COQ9, mitochondrialHomo sapiens (human)
Electron transfer flavoprotein subunit alpha, mitochondrialAlpha-ETFHomo sapiens (human)
All trans-polyprenyl-diphosphate synthase PDSS1All-trans-decaprenyl-diphosphate synthase subunit 1; 2.5.1.91; Decaprenyl pyrophosphate synthase subunit 1; Decaprenyl-diphosphate synthase subunit 1; Solanesyl-diphosphate synthase subunit 1; Trans-prenyltransferase 1; TPT 1Homo sapiens (human)
All trans-polyprenyl-diphosphate synthase PDSS2All-trans-decaprenyl-diphosphate synthase subunit 2; 2.5.1.91; Candidate tumor suppressor protein; Decaprenyl pyrophosphate synthase subunit 2; Decaprenyl-diphosphate synthase subunit 2; Solanesyl-diphosphate synthase subunit 2Homo sapiens (human)
4-hydroxybenzoate polyprenyltransferase, mitochondrial 4-HB polyprenyltransferase; EC 2.5.1.39; 4-hydroxybenzoate decaprenyltransferase; COQ2 homolog; hCOQ2; Para-hydroxybenzoate--polyprenyltransferase; PHB:PPT; PHB:polyprenyltransferaseHomo sapiens (human)
Solute carrier family 52, riboflavin transporter, member 2Porcine endogenous retrovirus A receptor 1; PERV-A receptor 1; Protein GPR172A; Riboflavin transporter 3; hRFT3Homo sapiens (human)
Solute carrier family 52, riboflavin transporter, member 1Porcine endogenous retrovirus A receptor 2; PERV-A receptor 2; huPAR-2; Protein GPR172B; Riboflavin transporter 1; hRFT1Homo sapiens (human)
Ubiquinone biosynthesis monooxygenase COQ6, mitochondrialEC 1.14.13.-; Coenzyme Q10 monooxygenase 6Homo sapiens (human)

Compounds (18)

CompoundDescription
diphosphoric acid
ironA metallic element with atomic symbol Fe, atomic number 26, and atomic weight 55.85. It is an essential constituent of HEMOGLOBINS; CYTOCHROMES; and IRON-BINDING PROTEINS. It plays a role in cellular redox reactions and in the transport of OXYGEN.
Flavin MononucleotideA coenzyme for a number of oxidative enzymes including NADH DEHYDROGENASE. It is the principal form in which RIBOFLAVIN is found in cells and tissues.
ubiquinol 1
riboflavinNutritional factor found in milk, eggs, malted barley, liver, kidney, heart, and leafy vegetables. The richest natural source is yeast. It occurs in the free form only in the retina of the eye, in whey, and in urine; its principal forms in tissues and cells are as FLAVIN MONONUCLEOTIDE and FLAVIN-ADENINE DINUCLEOTIDE.
coenzyme q10Ubiquinone ring with a chain of 10 isoprene units; redox equilibrium with ubiqunol serving in mitochondrial inner membrane to transfer electrons; presence during reconstitution of acetylcholine receptor into phospholipid vesicles yields vesicles active in catalyzing carbamylcholine-sensitive Na+ flux; coenzyme Q10 depletion has been noted with use of statins
adenosine monophosphateAdenine nucleotide containing one phosphate group esterified to the sugar moiety in the 2'-, 3'-, or 5'-position.
flavin-adenine dinucleotideA condensation product of riboflavin and adenosine diphosphate. The coenzyme of various aerobic dehydrogenases, e.g., D-amino acid oxidase and L-amino acid oxidase. (Lehninger, Principles of Biochemistry, 1982, p972)
flavin mononucleotideA coenzyme for a number of oxidative enzymes including NADH DEHYDROGENASE. It is the principal form in which RIBOFLAVIN is found in cells and tissues.
ubiquinolreduced forms of ubiquinone; see also record for ubiquinol 10
1,5-dihydro-FADchromophore component of E coli DNA photolyase
4-hydroxybenzoic acid
isopentenyl pyrophosphatesubstrate for isopentenyl pyrophosphate isomerase; RN given refers to unlabeled cpd; a nonpeptide mycobacterial antigen that stimulates gamma delta T cells
farnesyl pyrophosphatea sesquiterpene that dimerizes to SQUALENE; RN given refers to cpd without isomeric designation
ubiquinone q1interacts with iron atom to form acceptor quinone complex
3-decaprenyl-4-hydroxybenzoateintermediate in biosynthesis of ubiquinone
solanesyl pyrophosphate
Flavin-Adenine DinucleotideA condensation product of riboflavin and adenosine diphosphate. The coenzyme of various aerobic dehydrogenases, e.g., D-amino acid oxidase and L-amino acid oxidase. (Lehninger, Principles of Biochemistry, 1982, p972)