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Entries
BBOX1:AscH-:Fe2+ dimer dioxygenates TEABT and 2OG to form CAR and SUCCA
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catalystActivity
[CatalystActivity:71111] gamma-butyrobetaine dioxygenase activity of BBOX1:AscH-:Fe2+ dimer [cytosol]
catalystActivityReference
[CatalystActivityReference:9644024] gamma-butyrobetaine dioxygenase activity of BBOX1:AscH-:Fe2+ dimer [cytosol] Crystal structure of human gamma-butyrobetaine hydroxylase
category
transition
compartment
[Compartment:70101] cytosol
dbId
71261
displayName
BBOX1:AscH-:Fe2+ dimer dioxygenates TEABT and 2OG to form CAR and SUCCA
eventOf
[Pathway:R-HSA-71262] Carnitine synthesis - Homo sapiens
followingEvent
[BlackBoxEvent:R-HSA-164967] Unknown carnitine exporter transports CAR from the cytosol to the extracellular space - Homo sapiens
input
[SimpleEntity:R-ALL-29406] 2OG [cytosol]
[SimpleEntity:R-ALL-29368] O2 [cytosol]
[SimpleEntity:R-ALL-31369] TEABT [cytosol]
isInDisease
false
isInferred
false
literatureReference
[LiteratureReference:71108] Multiple forms of gamma-butyrobetaine hydroxylase (EC 1.14.11.1)
[LiteratureReference:9794599] Crystal structure of human gamma-butyrobetaine hydroxylase
[LiteratureReference:71105] Carnitine biosynthesis: identification of the cDNA encoding human gamma-butyrobetaine hydroxylase.
maxDepth
1
modified
[InstanceEdit:11080360] Weiser, Joel, 2026-03-16
name
BBOX1:AscH-:Fe2+ dimer dioxygenates TEABT and 2OG to form CAR and SUCCA
2-oxoglutarate + 4-(trimethylamino)butanoate + O2 => carnitine + CO2 + succinate
gamma-butyrobetaine + alpha-ketoglutarate + O2 => carnitine + succinate + CO2
orthologousEvent
[Reaction:R-SCE-71261] BBOX1:AscH-:Fe2+ dimer dioxygenates TEABT and 2OG to form CAR and SUCCA - Saccharomyces cerevisiae
[Reaction:R-CEL-71261] BBOX1:AscH-:Fe2+ dimer dioxygenates TEABT and 2OG to form CAR and SUCCA - Caenorhabditis elegans
[Reaction:R-DME-71261] BBOX1:AscH-:Fe2+ dimer dioxygenates TEABT and 2OG to form CAR and SUCCA - Drosophila melanogaster
[Reaction:R-GGA-71261] BBOX1:AscH-:Fe2+ dimer dioxygenates TEABT and 2OG to form CAR and SUCCA - Gallus gallus
[Reaction:R-XTR-71261] BBOX1:AscH-:Fe2+ dimer dioxygenates TEABT and 2OG to form CAR and SUCCA - Xenopus tropicalis
[Reaction:R-DRE-71261] BBOX1:AscH-:Fe2+ dimer dioxygenates TEABT and 2OG to form CAR and SUCCA - Danio rerio
[Reaction:R-SSC-71261] BBOX1:AscH-:Fe2+ dimer dioxygenates TEABT and 2OG to form CAR and SUCCA - Sus scrofa
[Reaction:R-BTA-71261] BBOX1:AscH-:Fe2+ dimer dioxygenates TEABT and 2OG to form CAR and SUCCA - Bos taurus
[Reaction:R-CFA-71261] BBOX1:AscH-:Fe2+ dimer dioxygenates TEABT and 2OG to form CAR and SUCCA - Canis familiaris
[Reaction:R-RNO-71261] BBOX1:AscH-:Fe2+ dimer dioxygenates TEABT and 2OG to form CAR and SUCCA - Rattus norvegicus
[Reaction:R-MMU-71261] BBOX1:AscH-:Fe2+ dimer dioxygenates TEABT and 2OG to form CAR and SUCCA - Mus musculus
output
[SimpleEntity:R-ALL-30235] CAR [cytosol]
[SimpleEntity:R-ALL-113528] CO2 [cytosol]
[SimpleEntity:R-ALL-29434] SUCCA [cytosol]
precedingEvent
[Reaction:R-HSA-71260] ALDH9A1 tetramer dehydrogenates TEABL to form TEABT - Homo sapiens
releaseDate
2004-07-06
reviewStatus
[ReviewStatus:9821382] five stars
revised
[InstanceEdit:9794704] D'Eustachio, Peter, 2022-07-19
schemaClass
Reaction
species
[Species:48887] Homo sapiens
stId
R-HSA-71261
summation
[Summation:178801] Cytosolic gamma-butyrobetaine hydroxylase dimer (BBOX1), a d...
updateTrackers
[UpdateTracker:9846949] Update Tracker - [Reaction:71261] BBOX1:AscH-:Fe2+ dimer dioxygenates TEABT and 2OG to form CAR and SUCCA - v82:[addLiteratureReference, modifyText]
[UpdateTracker:9781952] Update Tracker - [Reaction:71261] BBOX1:AscH-:Fe2+ dimer dioxygenates TEABT and 2OG to form CAR and SUCCA - v60:[modifyText]
[UpdateTracker:9789425] Update Tracker - [Reaction:71261] BBOX1:AscH-:Fe2+ dimer dioxygenates TEABT and 2OG to form CAR and SUCCA - v38:[modifyText]
Referrals
(updatedInstance)
[UpdateTracker:9846949] Update Tracker - [Reaction:71261] BBOX1:AscH-:Fe2+ dimer dioxygenates TEABT and 2OG to form CAR and SUCCA - v82:[addLiteratureReference, modifyText]
[UpdateTracker:9781952] Update Tracker - [Reaction:71261] BBOX1:AscH-:Fe2+ dimer dioxygenates TEABT and 2OG to form CAR and SUCCA - v60:[modifyText]
[UpdateTracker:9789425] Update Tracker - [Reaction:71261] BBOX1:AscH-:Fe2+ dimer dioxygenates TEABT and 2OG to form CAR and SUCCA - v38:[modifyText]
(hasEvent)
[Pathway:R-HSA-71262] Carnitine synthesis
(precedingEvent)
[BlackBoxEvent:R-HSA-164967] Unknown carnitine exporter transports CAR from the cytosol to the extracellular space
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