Ubiquinol biosynthesis

Stable Identifier
R-HSA-2142789
DOI
Type
Pathway
Species
Homo sapiens
ReviewStatus
5/5
Locations in the PathwayBrowser
General
SVG |   | PPTX  | SBGN
Click the image above or here to open this pathway in the Pathway Browser
Mitochondrial 4-hydroxyphenylpyruvate dioxygenase-like protein (HPDL) processes 4-hydroxphenylpyruvate (HPP, HPPA) to (S)-4-hydroxymandelate (4-HMA). HPDL defects lead to CoQ10 deficiency. The HPDL product 4-hydroxymandelate apparently is a precursor for the synthesis of 4-hydroxybenzoate, which is a prerequisite for the assembly of the CoQ10 head group (Banh et al., 2021). Because HPDL is a mitochondrial protein, cytosolic HPP from tyrosine catabolism must either be imported by a yet unknown transport mechanism, or mitochondrial HPP could be the product of an unknown mitochondrial reaction (Husain et al., 2020; reviewed in Staiano et al., 2023).
Literature References
PubMed ID Title Journal Year
19531029 Biosynthesis and bioproduction of coenzyme Q10 by yeasts and other organisms

Kawamukai, M

Biotechnol. Appl. Biochem. 2009
28552678 Detection of 6-demethoxyubiquinone in CoQ10 deficiency disorders: Insights into enzyme interactions and identification of potential therapeutics

Distelmaier, F, Wedell, A, Herebian, D, Freyer, C, Mayatepek, E, Seibt, A, Bünning, G, López, LC, Prokisch, H, Wredenberg, A, Smits, SHJ, Karall, D

Mol Genet Metab 2017
  Coenzyme Q: Molecular Mechanisms in Health and Disease

Quinn, PJ, Kagan, VE

  2000
17482885 Endogenous synthesis of coenzyme Q in eukaryotes

Tran, UC, Clarke, CF

Mitochondrion 2007
37508007 Biosynthesis, Deficiency, and Supplementation of Coenzyme Q

Brea-Calvo, G, Staiano, C, Mantle, D, Millichap, LE, Hargreaves, I, García-Corzo, L, Turton, N

Antioxidants (Basel) 2023
2295606 Nonaprenyl-4-hydroxybenzoate transferase, an enzyme involved in ubiquinone biosynthesis, in the endoplasmic reticulum-Golgi system of rat liver

Dallner, G, Kalén, A, Chojnacki, T, Appelkvist, EL

J Biol Chem 1990
37507930 New Insights on the Uptake and Trafficking of Coenzyme Q

Guile, MD, Jain, A, Clarke, CF, Anderson, KA

Antioxidants (Basel) 2023
36702698 Coenzyme Q biochemistry and biosynthesis

Pagliarini, DJ, Guerra, RM

Trends Biochem Sci 2023
27499296 Mitochondrial Protein Interaction Mapping Identifies Regulators of Respiratory Chain Function

Stefely, JA, Taylor, RW, Dolan, BK, Westphall, MS, Wilkerson, EM, Veling, MT, Wrobel, RL, Kim, JP, Jochem, A, Cho, H, Coon, JJ, Xia, C, Ulbrich, A, Gromek, KA, Rensvold, JW, Bohl, SL, Beebe, ET, Pagliarini, DJ, Werner, KM, Alston, CL, Floyd, BJ, Prokisch, H, Kremer, LS, Minogue, CE

Mol Cell 2016
11051212 Ubiquinone. Biosynthesis of quinone ring and its isoprenoid side chain. Intracellular localization

Szkopinska, A

Acta Biochim Pol 2000
28790927 The Role of Sulfide Oxidation Impairment in the Pathogenesis of Primary CoQ Deficiency

Luna-Sánchez, M, Quinzii, CM, Kleiner, G, López, LC, Hidalgo-Gutierrez, A, Ziosi, M

Front Physiol 2017
26183239 Biosynthesis of coenzyme Q in eukaryotes

Kawamukai, M

Biosci Biotechnol Biochem 2016
Participants
Participates
Event Information
Orthologous Events
Authored
Reviewed
Created
Cite Us!