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)).

Authors

Denise Slenter , Egon Willighagen , Finterly Hu , and Friederike Ehrhart

Cited In

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Organism

Homo sapiens

Communities

Inherited Metabolic Disorders (IMD) Pathways Rare Diseases

Annotations

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

Pathway Ontology: ubiquinone biosynthetic pathway riboflavin metabolic pathway

Participants

References

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