Primary Coenzyme Q10 Deficiency: An Update
The mammalian CoQ10 biosynthetic pathway with genes harbouring known pathogenic mutations associated with primary CoQ10 deficiency shown in red

Publication
Primary Coenzyme Q10 Deficiency: An Update (2023) David Mantle, et al. Antioxidants (Basel). 2023 Aug;12(8). Figure: F1.Organism | Group | Word | Match | Source | NCBI Symbol | NCBI ID |
---|---|---|---|---|---|---|
Homo sapiens | Primates | COQ2 | COQ2 | ncbigene_symbol | COQ2 | 27235 |
Homo sapiens | Primates | PDSS1 | PDSS1 | ncbigene_symbol | PDSS1 | 23590 |
Homo sapiens | Primates | PDSS2 | PDSS2 | ncbigene_symbol | PDSS2 | 57107 |
Homo sapiens | Primates | COQ4 | COQ4 | ncbigene_symbol | COQ4 | 51117 |
Homo sapiens | Primates | COQ3,5,7 | COQ3 | ncbigene_symbol | COQ3 | 51805 |
Homo sapiens | Primates | COQ3,5,7 | COQ5 | ncbigene_symbol | COQ5 | 84274 |
Homo sapiens | Primates | COQ3,5,7 | COQ7 | ncbigene_symbol | COQ7 | 10229 |
Homo sapiens | Primates | COQ8A | COQ8A | ncbigene_symbol | COQ8A | 56997 |
Homo sapiens | Primates | COQ9 | COQ9 | ncbigene_symbol | COQ9 | 57017 |
Homo sapiens | Primates | COQ10A | COQ10A | ncbigene_symbol | COQ10A | 93058 |
Homo sapiens | Primates | COQ10B | COQ10B | ncbigene_symbol | COQ10B | 80219 |
Homo sapiens | Primates | COQ6 | COQ6 | ncbigene_symbol | COQ6 | 51004 |
Homo sapiens | Primates | COQ8B | COQ8B | ncbigene_symbol | COQ8B | 79934 |
Homo sapiens | Primates | Q10 | Q10 | ncbigene_synonym | AGO2 | 27161 |
Word | Match | MeSH | Name | ChEBI |
---|---|---|---|---|
phenylalanine | phenylalanine | (2S)-2-azaniumyl-3-phenylpropanoate | chebi:58095 | |
4-hydroxybenzoate | 4-hydroxybenzoate | 4-hydroxybenzoate | chebi:17879 | |
pyrophosphate | Pyrophosphate | 1,5-dihydrido-2,4-dihydroxido-2,4-dioxido-1,3,5-trioxy-2,4-diphosphy-[5]catena | chebi:29888 | |
acetyl-CoA | acetyl-CoA | 3'-phosphonatoadenosine 5'-(3-{(3R)-4-[(3-{[2-(acetylsulfanyl)ethyl]amino}-3-oxopropyl)amino]-3-hydroxy-2,2-dimethyl-4-oxobutyl} diphosphate) | chebi:57288 | |
hydroxymethyl | hydroxymethyl | hydroxymethyl | chebi:24712 | |
glutaryl-CoA | glutaryl-CoA | 3'-phosphonatoadenosine 5'-{3-[(3R)-4-{[3-({2-[(4-carboxylatobutanoyl)sulfanyl]ethyl}amino)-3-oxopropyl]amino}-3-hydroxy-2,2-dimethyl-4-oxobutyl] diphosphate} | chebi:57378 | |
diphosphate | diphosphate | diphosphate | chebi:33019 | |
pyrophosphate | Pyrophosphate | 1,5-dihydrido-2,4-dihydroxido-2,4-dioxido-1,3,5-trioxy-2,4-diphosphy-[5]catena | chebi:29888 | |
COQ9 | CoQ9 | ubiquinone-9 | chebi:18160 | |
COQ5 | CoQ5 | ubiquinone-5 | chebi:46331 | |
COQ6 | CoQ6 | 2-[(2E,6E,10E,14E,18E)-3,7,11,15,19,23-hexamethyltetracosa-2,6,10,14,18,22-hexaen-1-yl]-5,6-dimethoxy-3-methylcyclohexa-2,5-diene-1,4-dione | chebi:52971 | |
COQ7 | CoQ7 | ubiquinone-7 | chebi:46448 | |
coenzyme | coenzyme | coenzyme | chebi:23354 | |
Q10 | Q10 | ubiquinone-10 | chebi:46245 | |
pyrophosphate | Pyrophosphate | 1,5-dihydrido-2,4-dihydroxido-2,4-dioxido-1,3,5-trioxy-2,4-diphosphy-[5]catena | chebi:29888 |
Disease mentions
Word | Match | MeSH | Name | DOID |
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