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Information on EC 2.3.1.61 - dihydrolipoyllysine-residue succinyltransferase and Organism(s) Mus musculus and UniProt Accession Q9D2G2

for references in articles please use BRENDA:EC2.3.1.61
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EC Tree
IUBMB Comments
A multimer (24-mer) of this enzyme forms the core of the multienzyme complex, and binds tightly both EC 1.2.4.2, oxoglutarate dehydrogenase (succinyl-transferring) and EC 1.8.1.4, dihydrolipoyl dehydrogenase. The lipoyl group of this enzyme is reductively succinylated by EC 1.2.4.2, and the only observed direction catalysed by EC 2.3.1.61 is that where this succinyl group is passed to coenzyme A.
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This record set is specific for:
Mus musculus
UNIPROT: Q9D2G2
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Word Map
The taxonomic range for the selected organisms is: Mus musculus
The expected taxonomic range for this enzyme is: Bacteria, Eukaryota, Archaea
Synonyms
dihydrolipoamide succinyltransferase, ogdc-e2, dihydrolipoyl succinyltransferase, dihydrolipoamide s-succinyltransferase, dihydrolipoyl transsuccinylase, lipoate succinyltransferase, dihydrolipoyllysine-residue succinyltransferase, dihydrolipoyl succinyl transferase, e2 component of alpha-ketoglutarate dehydrogenase complex, lipoyl transsuccinylase, more
SYNONYM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
dihydrolipolyl succinyltransferase
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E2 subcomponent of the alpha-ketoglutarate dehydrogenase complex
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dihydrolipoamide succinyltransferase
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-
-
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dihydrolipoic transsuccinylase
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-
-
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dihydrolipolyl transsuccinylase
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-
-
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dihydrolipoyl succinyl transferase
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dihydrolipoyl transsuccinylase
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-
-
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DLST
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lipoate succinyltransferase
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-
-
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lipoic transsuccinylase
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-
-
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lipoyl transsuccinylase
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-
-
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succinyl-CoA:dihydrolipoate S-succinyltransferase
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-
-
-
REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
Acyl group transfer
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-
-
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oxidative decarboxylation
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-
-
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SYSTEMATIC NAME
IUBMB Comments
succinyl-CoA:enzyme-N6-(dihydrolipoyl)lysine S-succinyltransferase
A multimer (24-mer) of this enzyme forms the core of the multienzyme complex, and binds tightly both EC 1.2.4.2, oxoglutarate dehydrogenase (succinyl-transferring) and EC 1.8.1.4, dihydrolipoyl dehydrogenase. The lipoyl group of this enzyme is reductively succinylated by EC 1.2.4.2, and the only observed direction catalysed by EC 2.3.1.61 is that where this succinyl group is passed to coenzyme A.
CAS REGISTRY NUMBER
COMMENTARY hide
9032-28-4
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SUBSTRATE
PRODUCT                       
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
succinyl-CoA + enzyme N6-(dihydrolipoyl)lysine
CoA + enzyme N6-(S-succinyldihydrolipoyl)lysine
show the reaction diagram
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-
-
?
NATURAL SUBSTRATE
NATURAL PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
succinyl-CoA + enzyme N6-(dihydrolipoyl)lysine
CoA + enzyme N6-(S-succinyldihydrolipoyl)lysine
show the reaction diagram
-
-
-
?
ORGANISM
COMMENTARY hide
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
-
UniProt
Manually annotated by BRENDA team
SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
SOURCE
LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY hide
GeneOntology No.
LITERATURE
SOURCE
GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
physiological function
enzyme overexpression in wild type mice protects against cardiac hypertrophy and dysfunction in vivo
malfunction
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enzyme deficient mice show reduced mRNA and protein levels and decreased brain mitochondrial alpha-ketoglutarate dehydrogenase activity (by about 40%), increased vulnerability to mitochondrial toxins: MPTP treatment enhances the severity of lipid peroxidation in the substantial nigra, reduced striatal dopamine (59% depletion in wild-type, 73% in enzyme deficient mutants), dopaminergic neurons (25% reduction in wild-type, 42% in mutants) and tyrosine hydroxylase-positive neurons, striatal lesions induced by malonate (mimicking Huntington's disease, 2fold larger lesions, smaller striatum) or 3-nitropropionic acid (5fold larger lesions than in wild-type) are significantly larger in enzyme deficient mice than in the wild-type, and the 3-nitropropionic acid-induced mitochondrial enzyme inhibition (25% lower citrate synthase activity than in wild-type), protein and DNA oxidation is enhanced in the cortex of enzyme deficient mice compared to wild-type
physiological function
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subunit of the alpha-ketoglutarate dehydrogenase complex
UNIPROT
ENTRY NAME
ORGANISM
NO. OF AA
NO. OF TRANSM. HELICES
MOLECULAR WEIGHT[Da]
SOURCE
SEQUENCE
LOCALIZATION PREDICTION?
ODO2_MOUSE
454
0
48995
Swiss-Prot
Mitochondrion (Reliability: 2)
EXPRESSION
ORGANISM
UNIPROT
LITERATURE
enzyme protein levels significantly decrease in wild type mice after 4 weeks of angiotensin-II or 6 weeks of transversal aortic constriction. Enzyme levels significantly decrease in microRNA-146a wild-type mice on angiotensin-II administration
REF.
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
Yang, L.; Shi, Q.; Ho, D.J.; Starkov, A.A.; Wille, E.J.; Xu, H.; Chen, H.L.; Zhang, S.; Stack, C.M.; Calingasan, N.Y.; Gibson, G.E.; Beal, M.F.
Mice deficient in dihydrolipoyl succinyl transferase show increased vulnerability to mitochondrial toxins
Neurobiol. Dis.
36
320-330
2009
Mus musculus
Manually annotated by BRENDA team
Heggermont, W.A.; Papageorgiou, A.P.; Quaegebeur, A.; Deckx, S.; Carai, P.; Verhesen, W.; Eelen, G.; Schoors, S.; van Leeuwen, R.; Alekseev, S.; Elzenaar, I.; Vinckier, S.; Pokreisz, P.; Walravens, A.S.; Gijsbers, R.; Van Den Haute, C.; Nickel, A.; Schroen, B.; van Bilsen, M.; Janssens, S.; Maack, C.; Pinto, Y.; Carmeliet, P.; Heymans, S.
Inhibition of microRNA-146a and overexpression of its target dihydrolipoyl succinyltransferase protect against pressure overload-induced cardiac hypertrophy and dysfunction
Circulation
136
747-761
2017
Mus musculus (Q9D2G2), Mus musculus
Manually annotated by BRENDA team