Riboflavin and CoQ disorders (WP5037)

Riboflavin (aka Vitamin B2) is used as molecular precursor for the formation of FAD and FMN, which are both essential cofactors in beta-oxidation, branched-chain-amino-acid catabolism and the mitochondrial electron transport of the TCA cycle. Coenzyme Q10 (aka ubiquinone or CoQ10) functions as a electron carrier, antioxidant and influences pyrimidine metabolism directly. This pathway was inspired by Chapter 16 (ed. 4) from the book of Blau (ISBN 3642403360 (978-3642403361)).
last edited


DeSl, Egonw, Finterly, and Fehrhart

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Homo sapiens


Inborn Errors of Metabolism (IEM) Pathways Rare Diseases


Disease Ontology: primary coenzyme Q10 deficiency 2 Brown-Vialetto-Van Laere syndrome Fazio-Londe disease primary coenzyme Q10 deficiency 3 primary coenzyme Q10 deficiency 1 coenzyme Q10 deficiency disease primary coenzyme Q10 deficiency 4 riboflavin deficiency Brown-Vialetto-Van Laere syndrome 2 primary coenzyme Q10 deficiency 6 primary coenzyme Q10 deficiency 5 Brown-Vialetto-Van Laere syndrome 1

Pathway Ontology: riboflavin metabolic pathway ubiquinone biosynthetic pathway


Label Type Compact Identifier
One carbonmetabolism Pathway wikipathways:WP241
a ubiquinone Metabolite chebi:16389
Mevalonatepathway Pathway wikipathways:WP3963
PDSS1 Protein uniprot:Q5T2R2
Riboflavin kinase Protein uniprot:Q969G6
Pyrimidine metabolismand related disorders Pathway wikipathways:WP4225
FADH2 Metabolite chebi:58307
CABC1 Protein uniprot:Q8NI60
6 PP Metabolite chebi:33019
OXPHOS Pathway wikipathways:WP111
FMN Metabolite chebi:58210
APTX Protein uniprot:Q7Z2E3
trans-decaprenyl-PP Metabolite chebi:60721
CETF GeneProduct interpro:IPR014730
FAD synthetase Protein uniprot:Q8NFF5
CoQ10 Metabolite chebi:46245
ETFDH gene GeneProduct ensembl:ENSG00000171503
6 IPP Metabolite chebi:128769
ETFDH Protein uniprot:Q16134
Farnesyl-PP Metabolite chebi:175763
AMP Metabolite chebi:16027
RIB Metabolite chebi:17015
SLC52A1 Protein uniprot:Q9NWF4
FMN Metabolite chebi:17621
RIB Metabolite chebi:17015
FAD Metabolite chebi:16238
SLC52A2 Protein uniprot:Q9HAB3
SLC52A3 Protein uniprot:SLC52A3
ETFA Protein uniprot:P13804
ETFB Protein uniprot:38117
FAD Metabolite chebi:57692
a ubiquinol Metabolite chebi:17976
FAD Metabolite chebi:57692
FAD Metabolite chebi:57692
PHB Metabolite chebi:30763
DHB Metabolite chebi:64136
all-E-10PrP2 Metabolite chebi:61011
COQ2 Protein uniprot:Q96H96
PPi Metabolite chebi:29888
FAD Metabolite chebi:16238
COQ6 Protein uniprot:Q9Y2Z9
DHDB Metabolite chebi:50775
Q10H2 Metabolite chebi:64183
DMQ10H2 Metabolite chebi:64181
DeMQ10H2 Metabolite chebi:64182
MHDB Metabolite chebi:50776
DMPhOH Metabolite chebi:50774
MDMQ10H2 Metabolite chebi:64180
COQ7 Protein uniprot:Q99807
Fe2+ Metabolite chebi:29033
COQ9 Protein uniprot:O75208
PDSS2 Protein uniprot:Q86YH6
trans-nonaprenyl-PP Metabolite chebi:58391
7 PP Metabolite chebi:33019
7 IPP Metabolite chebi:128769


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