Page last updated: 2024-10-06
Riboflavin and CoQ disorders
Proteins (14)
| Protein | Synonyms | Taxonomy |
|---|---|---|
| Aprataxin | EC 3.6.1.71; EC 3.6.1.72; Forkhead-associated domain histidine triad-like protein; FHA-HIT | Homo sapiens (human) |
| Electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial | ETF-QO; ETF-ubiquinone oxidoreductase; EC 1.5.5.1; Electron-transferring-flavoprotein dehydrogenase; ETF dehydrogenase | Homo sapiens (human) |
| 5-demethoxyubiquinone hydroxylase, mitochondrial | DMQ hydroxylase; EC 1.14.99.60; Timing protein clk-1 homolog; Ubiquinone biosynthesis monooxygenase COQ7 | Homo sapiens (human) |
| Atypical kinase COQ8A, mitochondrial | EC 2.7.-.-; Chaperone activity of bc1 complex-like; Chaperone-ABC1-like; Coenzyme Q protein 8A; aarF domain-containing protein kinase 3 | Homo sapiens (human) |
| FAD synthase | EC 2.7.7.2; FAD pyrophosphorylase; FMN adenylyltransferase; Flavin adenine dinucleotide synthase | Homo sapiens (human) |
| Riboflavin kinase | EC 2.7.1.26; ATP:riboflavin 5'-phosphotransferase; Flavokinase | Homo sapiens (human) |
| Ubiquinone biosynthesis protein COQ9, mitochondrial | Homo sapiens (human) | |
| Electron transfer flavoprotein subunit alpha, mitochondrial | Alpha-ETF | Homo sapiens (human) |
| All trans-polyprenyl-diphosphate synthase PDSS1 | All-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 1 | Homo sapiens (human) |
| All trans-polyprenyl-diphosphate synthase PDSS2 | All-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 2 | Homo 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:polyprenyltransferase | Homo sapiens (human) |
| Solute carrier family 52, riboflavin transporter, member 2 | Porcine endogenous retrovirus A receptor 1; PERV-A receptor 1; Protein GPR172A; Riboflavin transporter 3; hRFT3 | Homo sapiens (human) |
| Solute carrier family 52, riboflavin transporter, member 1 | Porcine endogenous retrovirus A receptor 2; PERV-A receptor 2; huPAR-2; Protein GPR172B; Riboflavin transporter 1; hRFT1 | Homo sapiens (human) |
| Ubiquinone biosynthesis monooxygenase COQ6, mitochondrial | EC 1.14.13.-; Coenzyme Q10 monooxygenase 6 | Homo sapiens (human) |
Compounds (18)
| Compound | Description |
|---|---|
| diphosphoric acid | |
| iron | A 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 Mononucleotide | A 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 | |
| riboflavin | Nutritional 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 q10 | Ubiquinone 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 monophosphate | Adenine nucleotide containing one phosphate group esterified to the sugar moiety in the 2'-, 3'-, or 5'-position. |
| flavin-adenine dinucleotide | A 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 mononucleotide | A 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 | reduced forms of ubiquinone; see also record for ubiquinol 10 |
| 1,5-dihydro-FAD | chromophore component of E coli DNA photolyase |
| 4-hydroxybenzoic acid | |
| isopentenyl pyrophosphate | substrate for isopentenyl pyrophosphate isomerase; RN given refers to unlabeled cpd; a nonpeptide mycobacterial antigen that stimulates gamma delta T cells |
| farnesyl pyrophosphate | a sesquiterpene that dimerizes to SQUALENE; RN given refers to cpd without isomeric designation |
| ubiquinone q1 | interacts with iron atom to form acceptor quinone complex |
| 3-decaprenyl-4-hydroxybenzoate | intermediate in biosynthesis of ubiquinone |
| solanesyl pyrophosphate | |
| Flavin-Adenine Dinucleotide | A 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) |