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Entries
SUCLG1/A2 cleaves succinyl-CoA
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catalystActivity
[CatalystActivity:70996] succinate-CoA ligase (ADP-forming) activity of SUCLA2:SUCLG1 [mitochondrial matrix]
catalystActivityReference
[CatalystActivityReference:9643157] succinate-CoA ligase (ADP-forming) activity of SUCLA2:SUCLG1 [mitochondrial matrix] Deficiency of the ADP-forming succinyl-CoA synthase activity is associated with encephalomyopathy and mitochondrial DNA depletion
category
transition
compartment
[Compartment:5460] mitochondrial matrix
crossReference
[DatabaseIdentifier:12610604] Rhea:17663
[DatabaseIdentifier:9854184] RHEA:17662
dbId
70997
displayName
SUCLG1/A2 cleaves succinyl-CoA
edited
[InstanceEdit:165080] D'Eustachio, P, 0000-00-00 00:00:00
eventOf
[Pathway:R-HSA-71403] Citric acid cycle (TCA cycle) - Homo sapiens
followingEvent
[Reaction:R-HSA-163213] Transfer of electrons through the succinate dehydrogenase complex - Homo sapiens
[Reaction:R-HSA-70994] SDH complex dehydrogenates succinate - Homo sapiens
input
[SimpleEntity:R-ALL-113581] ADP [mitochondrial matrix]
[SimpleEntity:R-ALL-113548] Pi [mitochondrial matrix]
[SimpleEntity:R-ALL-70958] SUCC-CoA [mitochondrial matrix]
isInDisease
false
isInferred
false
literatureReference
[LiteratureReference:451032] Deficiency of the ADP-forming succinyl-CoA synthase activity is associated with encephalomyopathy and mitochondrial DNA depletion
[LiteratureReference:156639] Interaction between succinyl CoA synthetase and the heme-biosynthetic enzyme ALAS-E is disrupted in sideroblastic anemia
[LiteratureReference:156645] Genetic evidence for the expression of ATP- and GTP-specific succinyl-CoA synthetases in multicellular eucaryotes
[LiteratureReference:156641] Characterization of the ATP- and GTP-specific succinyl-CoA synthetases in pigeon. The enzymes incorporate the same alpha-subunit.
[LiteratureReference:156646] Expression of two succinyl-CoA synthetases with different nucleotide specificities in mammalian tissues
[LiteratureReference:156637] Guanosine triphosphate, the primary product of phosphorylation coupled to the breakdown of succinyl coenzyme A
[LiteratureReference:9853989] SUCLG1 mutations and mitochondrial encephalomyopathy: a case study and review of the literature
maxDepth
1
modified
[InstanceEdit:11064438] Weiser, Joel, 2025年08月28日
name
SUCLG1/A2 cleaves succinyl-CoA
ADP + phosphate + succinyl-CoA => ATP + CoA + succinate
negativePrecedingEvent
[NegativePrecedingEvent:9908922] Futile Cycle: DLST transfers succinyl to CoA
orthologousEvent
[Reaction:R-DRE-70997] SUCLG1/A2 cleaves succinyl-CoA - Danio rerio
[Reaction:R-RNO-70997] SUCLG1/A2 cleaves succinyl-CoA - Rattus norvegicus
[Reaction:R-PFA-70997] SUCLG1/A2 cleaves succinyl-CoA - Plasmodium falciparum
[Reaction:R-XTR-70997] SUCLG1/A2 cleaves succinyl-CoA - Xenopus tropicalis
[Reaction:R-DDI-70997] SUCLG1/A2 cleaves succinyl-CoA - Dictyostelium discoideum
[Reaction:R-CFA-70997] SUCLG1/A2 cleaves succinyl-CoA - Canis familiaris
[Reaction:R-DME-70997] SUCLG1/A2 cleaves succinyl-CoA - Drosophila melanogaster
[Reaction:R-GGA-70997] SUCLG1/A2 cleaves succinyl-CoA - Gallus gallus
[Reaction:R-SCE-70997] SUCLG1/A2 cleaves succinyl-CoA - Saccharomyces cerevisiae
[Reaction:R-SPO-70997] SUCLG1/A2 cleaves succinyl-CoA - Schizosaccharomyces pombe
[Reaction:R-CEL-70997] SUCLG1/A2 cleaves succinyl-CoA - Caenorhabditis elegans
[Reaction:R-MMU-70997] SUCLG1/A2 cleaves succinyl-CoA - Mus musculus
[Reaction:R-SSC-70997] SUCLG1/A2 cleaves succinyl-CoA - Sus scrofa
[Reaction:R-BTA-70997] SUCLG1/A2 cleaves succinyl-CoA - Bos taurus
output
[SimpleEntity:R-ALL-113593] ATP [mitochondrial matrix]
[SimpleEntity:R-ALL-29374] CoA-SH [mitochondrial matrix]
[SimpleEntity:R-ALL-113536] SUCCA [mitochondrial matrix]
precedingEvent
[Reaction:R-HSA-71401] OGDH dimer decarboxylates 2-OG - Homo sapiens
[Reaction:R-HSA-71010] MUT isomerises L-MM-CoA to SUCC-CoA - Homo sapiens
releaseDate
2004年07月06日
reviewStatus
[ReviewStatus:9821382] five stars
reviewed
[InstanceEdit:9861215] Hill, David P, 2024年02月15日
revised
[InstanceEdit:451045] D'Eustachio, P, 2009年12月26日
schemaClass
Reaction
species
[Species:48887] Homo sapiens
stId
R-HSA-70997
summation
[Summation:71035] Mitochondrial succinate CoA ligase (ADP-forming) catalyzes t...
updateTrackers
[UpdateTracker:9791122] Update Tracker - [Reaction:70997] ADP + Orthophosphate + Succinyl-CoA <=> ATP + Succinate + CoA - v32:[addLiteratureReference, modifyText]
[UpdateTracker:9864283] Update Tracker - [Reaction:70997] SUCLG1/A2 cleaves succinyl-CoA - v88:[addLiteratureReference, modifyText]
Referrals
(updatedInstance)
[UpdateTracker:9791122] Update Tracker - [Reaction:70997] ADP + Orthophosphate + Succinyl-CoA <=> ATP + Succinate + CoA - v32:[addLiteratureReference, modifyText]
[UpdateTracker:9864283] Update Tracker - [Reaction:70997] SUCLG1/A2 cleaves succinyl-CoA - v88:[addLiteratureReference, modifyText]
(hasEvent)
[Pathway:R-HSA-71403] Citric acid cycle (TCA cycle)
(precedingEvent)
[Reaction:R-HSA-163213] Transfer of electrons through the succinate dehydrogenase complex
[Reaction:R-HSA-70994] SDH complex dehydrogenates succinate
[NegativePrecedingEvent:9908924] Futile Cycle: SUCLG1/A2 cleaves succinyl-CoA
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