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Details on Person ornithine (cytosolic) + citrulline (mitochondrial) => ornithine (mitochondrial) + citrulline (cytosolic)
Class:Id Reaction:70634
_displayName ornithine (cytosolic) + citrulline (mitochondrial) => ornithine (mitochondrial) + citrulline (cytosolic)
_doRelease TRUE
_timestamp 2025-12-31 17:58:50
authored [InstanceEdit:73677] D'Eustachio, P, 2003-06-24 00:00:00
catalystActivity [CatalystActivity:70633] L-ornithine transmembrane transporter activity of Mitochondrial ornithine transporters [mitochondrial inner membrane]
catalystActivityReference [CatalystActivityReference:9643582] L-ornithine transmembrane transporter activity of Mitochondrial ornithine transporters [mitochondrial inner membrane] The mitochondrial ornithine transporter. Bacterial expression, reconstitution, functional characterization, and tissue distribution of two human isoforms
compartment [Compartment:70101] cytosol [Compartment:70616] mitochondrial inner membrane [Compartment:5460] mitochondrial matrix
crossReference [DatabaseIdentifier:9978418] RHEA:70787
edited [InstanceEdit:73677] D'Eustachio, P, 2003-06-24 00:00:00
input [SimpleEntity:113567] L-Cit [mitochondrial matrix] [SimpleEntity:29498] L-Orn [cytosol] [SimpleEntity:113529] H+ [mitochondrial matrix]
internalReviewed [InstanceEdit:9978421] D'Eustachio, Peter, 2025-12-31
literatureReference [LiteratureReference:70619] Hyperornithinaemia-hyperammonaemia-homocitrullinuria syndrome is caused by mutations in a gene encoding a mitochondrial ornithine transporter [LiteratureReference:374019] The mitochondrial ornithine transporter. Bacterial expression, reconstitution, functional characterization, and tissue distribution of two human isoforms [LiteratureReference:449686] Cloning and characterization of human ORNT2: a second mitochondrial ornithine transporter that can rescue a defective ORNT1 in patients with the hyperornithinemia-hyperammonemia-homocitrullinuria syndrome, a urea cycle disorder
modified [InstanceEdit:113652] Schmidt, EE, 2004-06-11 08:58:20 [InstanceEdit:163376] Vastrik, I, 2005-04-29 06:26:51 [InstanceEdit:374021] D'Eustachio, P, 2008-07-24 20:44:45 [InstanceEdit:374024] D'Eustachio, P, 2008-07-24 21:06:09 [InstanceEdit:449690] D'Eustachio, P, 2009-12-05 [InstanceEdit:452030] D'Eustachio, P, 2010-01-14 [InstanceEdit:517476] D'Eustachio, P, 2010-02-18 [InstanceEdit:1080818] D'Eustachio, P, 2010-11-27 [InstanceEdit:1667321] D'Eustachio, P, 2011-10-14 [InstanceEdit:8946993] Shorser, Solomon, 2016-11-07 [InstanceEdit:9643047] Wu, G, 2019-04-04 [InstanceEdit:9830342] Matthews, Lisa, 2023-03-08 [InstanceEdit:9978417] Gupta, Parul, 2025-12-31 [InstanceEdit:9978420] Gupta, Parul, 2025-12-31 [InstanceEdit:9978424] Gupta, Parul, 2025-12-31
name ornithine (cytosolic) + citrulline (mitochondrial) => ornithine (mitochondrial) + citrulline (cytosolic) L-citrulline(in) + L-ornithine(out) + H(+)(in) = L-citrulline(out) + L-ornithine(in) + H(+)(out)
output [SimpleEntity:29968] L-Cit [cytosol] [SimpleEntity:113585] L-Orn [mitochondrial matrix] [SimpleEntity:70106] H+ [cytosol]
precedingEvent
previousReviewStatus [ReviewStatus:9821385] four stars
releaseDate 2004-07-06
reviewed [InstanceEdit:9978423] D'Eustachio, Peter, 2025-12-31
reviewStatus [ReviewStatus:9821382] five stars
species [Species:48887] Homo sapiens
stableIdentifier [StableIdentifier:359721] R-HSA-70634.5
structureModified [InstanceEdit:9978417] Gupta, Parul, 2025-12-31
summation [Summation:179015] The mitochondrial ornithine transporters SLC25A15 and SLC25A...
(hasEvent) [Pathway:70635] Urea cycle [Homo sapiens]
(inferredFrom) [Reaction:187608] ornithine (cytosolic) + citrulline (mitochondrial) => ornithine (mitochondrial) + citrulline (cytosolic) [Gallus gallus]
(normalReaction) [FailedReaction:9956519] SLC25A15 variants don't translocate ornithine and citrulline [Homo sapiens]
(precedingEvent) [Reaction:70560] carbamoyl phosphate + ornithine => citrulline + orthophosphate [Homo sapiens] [Reaction:70577] ASS1 tetramer:NMRAL1 dimer:NADPH transforms L-Asp and L-Cit to ARSUA [Homo sapiens] [Reaction:70654] ornithine + alpha-ketoglutarate <=> glutamate + L-glutamate gamma-semialdehyde [OAT] [Homo sapiens]
(updatedInstance) [_UpdateTracker:9791003] Update Tracker - [Reaction:70634] ornithine (cytosolic) + citrulline (mitochondrial) => ornithine (mitochondrial) + citrulline (cytosolic) - v32:[addLiteratureReference] [_UpdateTracker:9791769] Update Tracker - [Reaction:70634] ornithine (cytosolic) + citrulline (mitochondrial) => ornithine (mitochondrial) + citrulline (cytosolic) - v26:[add_removeLiteratureReference, modifyText] [_UpdateTracker:9984648] Update Tracker - [Reaction:70634] ornithine (cytosolic) + citrulline (mitochondrial) => ornithine (mitochondrial) + citrulline (cytosolic) - v96:[removeInput, removeOutput]
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No pathways have been reviewed or authored by ornithine (cytosolic) + citrulline (mitochondrial) => ornithine (mitochondrial) + citrulline (cytosolic) (70634)