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Literature summary for 2.5.1.39 extracted from

  • Quinzii, C.M.; Tadesse, S.; Naini, A.; Hirano, M.
    Effects of inhibiting CoQ10 biosynthesis with 4-nitrobenzoate in human fibroblasts (2012), PLoS ONE, 7, e30606.
    View publication on PubMedView publication on EuropePMC

Inhibitors

Inhibitors Comment Organism Structure
4-nitrobenzoate inhibition of COQ2 by 4-nitrobenzoate, leading to 40-50% residual CoQ10, is associated with increased oxidative stress and reduced viability, together with moderately decreased ATP levels and ATP/ADP ratio Homo sapiens

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
gene COQ2
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Source Tissue

Source Tissue Comment Organism Textmining
fibroblast
-
Homo sapiens
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
additional information decaprenyl diphosphate and 4-hydroxybenzoate are condensed in a reaction catalyzed by 4-hydroxybenzoate:polyprenyl transferase, i.e. COQ2 Homo sapiens ?
-
?

Synonyms

Synonyms Comment Organism
4-para-hydroxybenzoate:polyprenyl transferase
-
Homo sapiens
COQ2
-
Homo sapiens

General Information

General Information Comment Organism
malfunction cell viability in skin fibroblasts with CoQ10 deficiency due to different molecular defects including mutations in COQ2. Treatment of multiple cell lines with increasing dosages of 4-nitrobenzoate, which inhibits 4-hydroxybenzoate:polyprenyltransferase, leads to dose-dependent decreases of CoQ in mammalian cells without directly inducing oxidative stress or mitochondrial respiration impairment. Fibroblasts from a patient with a homozygous COQ2 mutation require uridine to maintain cell growth and proposed that deficiency of CoQ10 impaired pyrimidine biosynthesis due to dependence of dihydro-orotate dehydrogenase on ubiquinol. Oxidative stress plays an important role in the demise of COQ2 mutant fibroblasts by activating cell-death related pathways, which are averted by antioxidant supplementation Homo sapiens
metabolism the enzyme catalyzes one of the first steps in ubiquinone or coenzyme Q, CoQ, biosynthesis Homo sapiens