Reactome | COQ6 hydroxylates DHB

Schema > Reaction > Entries

COQ6 hydroxylates DHB Show undefined attributes

authored	[InstanceEdit:2162344] Williams, MG, 2012-03-19
catalystActivity	[CatalystActivity:2162332] oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, NAD(P)H as one donor, and incorporation of one atom of oxygen of COQ3:COQ4:COQ5:COQ6:COQ7:COQ9 [mitochondrial inner membrane]
category	transition
compartment	[Compartment:70616] mitochondrial inner membrane [Compartment:5460] mitochondrial matrix
created	[InstanceEdit:2162189] Williams, MG, 2012-03-15
dbId	2162187
displayName	COQ6 hydroxylates DHB
edited	[InstanceEdit:2167908] Williams, MG, 2012-03-23
eventOf	[Pathway:R-HSA-2142789] Ubiquinol biosynthesis - Homo sapiens
followingEvent	[Reaction:R-HSA-2162193] COQ3 methylates DHDB - Homo sapiens
inferredFrom	[Reaction:R-SCE-2162292] HHB is hydroxylated to DHHB by Coq6 - Saccharomyces cerevisiae
input	[SimpleEntity:R-ALL-2395504] (2Fe-2S)(1+) [mitochondrial matrix] [SimpleEntity:R-ALL-2162207] DHB [mitochondrial inner membrane] [SimpleEntity:R-ALL-113529] H+ [mitochondrial matrix] [SimpleEntity:R-ALL-113533] O2 [mitochondrial matrix]
isChimeric	false
isInDisease	false
isInferred	true
literatureReference	[LiteratureReference:2162291] COQ6 mutations in human patients produce nephrotic syndrome with sensorineural deafness [LiteratureReference:2162288] Coenzyme Q biosynthesis: Coq6 is required for the C5-hydroxylation reaction and substrate analogs rescue Coq6 deficiency [LiteratureReference:2162300] The Saccharomyces cerevisiae COQ6 gene encodes a mitochondrial flavin-dependent monooxygenase required for coenzyme Q biosynthesis [LiteratureReference:9856314] Mitochondrial Protein Interaction Mapping Identifies Regulators of Respiratory Chain Function [LiteratureReference:9856303] ADCK4 mutations promote steroid-resistant nephrotic syndrome through CoQ10 biosynthesis disruption [LiteratureReference:9856341] Adolescence Onset Primary Coenzyme Q10 Deficiency With Rare CoQ8A Gene Mutation: A Case Report and Review of Literature [LiteratureReference:9856349] Clinical features and gene variation analysis of COQ8B nephropathy: Report of seven cases
modified	[InstanceEdit:9857488] Stephan, Ralf, 2024-01-07
name	COQ6 hydroxylates DHB DHB is hydroxylated to DHDB by COQ6
output	[SimpleEntity:R-ALL-2395501] (2Fe-2S)2+ [mitochondrial matrix] [SimpleEntity:R-ALL-2162209] DHDB [mitochondrial inner membrane] [SimpleEntity:R-ALL-113521] H2O [mitochondrial matrix]
precedingEvent	[Reaction:R-HSA-2162192] COQ2 ligates all-E-10PrP2 to PHB - Homo sapiens
previousReviewStatus	[ReviewStatus:9821384] two stars
regulatedBy	[Requirement:9856293] Requirement by 'COQ8A,B:COQ6 [mitochondrial inner membrane]'
regulationReference	[RegulationReference:9856297] Requirement by 'COQ8A,B:COQ6 [mitochondrial inner membrane]' Mitochondrial Protein Interaction Mapping Identifies Regulators of Respiratory Chain Function
releaseDate	2013-03-12
reviewStatus	[ReviewStatus:9821385] four stars
reviewed	[InstanceEdit:3009435] Kawamukai, Makoto, 2013-02-05
schemaClass	Reaction
species	[Species:48887] Homo sapiens
stId	R-HSA-2162187
summation	[Summation:2167893] Flavin-dependent monooxygenase COQ6 (Heeringa et al. 2011) c...

Referrals

(hasEvent)	[Pathway:R-HSA-2142789] Ubiquinol biosynthesis
(precedingEvent)	[Reaction:R-HSA-2162193] COQ3 methylates DHDB
(inferredTo)	[Reaction:R-SCE-2162292] HHB is hydroxylated to DHHB by Coq6

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