Information on EC 2.3.1.61 - dihydrolipoyllysine-residue succinyltransferase and Organism(s) Homo sapiens

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The expected taxonomic range for this enzyme is: Bacteria, Eukaryota


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EC NUMBER
COMMENTARY hide
2.3.1.61
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RECOMMENDED NAME
GeneOntology No.
dihydrolipoyllysine-residue succinyltransferase
REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
Acyl group transfer
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oxidative decarboxylation
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PATHWAY
BRENDA Link
KEGG Link
MetaCyc Link
2-oxoglutarate decarboxylation to succinyl-CoA
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TCA cycle III (animals)
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citric acid cycle
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vitamin B1 metabolism
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Citrate cycle (TCA cycle)
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Lysine degradation
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Metabolic pathways
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Biosynthesis of secondary metabolites
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Microbial metabolism in diverse environments
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Biosynthesis of antibiotics
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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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ORGANISM
COMMENTARY hide
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
malfunction
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reduced enzyme activity increases H2O2-induced reactive oxygen species production and cell death, in human Alzheimer disease brains the activity of the alpha-ketoglutarate dehydrogenase enzyme complex is reduced, enzyme deficient mice show increased plaque burden, Abeta oligomers, and nitrotyrosine levels, the occurrence of spatial learning and memory deficits in female Tg19959 mice is accelerated
physiological function
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key subunit of the alpha-ketoglutarate dehydrogenase enzyme complex, involved in NADH production, participation in oxidative stress and reactive oxygen species (ROS) production
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 + dihydrolipoamide
CoA + S-succinyldihydrolipoamide
show the reaction diagram
additional information
?
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the DLST gene is bifunctionally involved in mitochondrial energy metabolism, the full-length DLST protein is involved in the Krebs cycle and the truncated version, MIRTD, contributes to the biogenesis of the respiratory chain. The genotype of the DLST gene is associated with Alztheimer‘s disease without a change of amino acid residues in the DLST protein: through a decrease in the expression of the truncated gene product MIRTD and the function of MIRTD. It remains unclear how much MIRTD actually contributes to the pathogenesis of Alzheimer‘s disease
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NATURAL SUBSTRATES
NATURAL PRODUCTS
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
succinyl-CoA + dihydrolipoamide
CoA + S-succinyldihydrolipoamide
show the reaction diagram
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?
additional information
?
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the DLST gene is bifunctionally involved in mitochondrial energy metabolism, the full-length DLST protein is involved in the Krebs cycle and the truncated version, MIRTD, contributes to the biogenesis of the respiratory chain. The genotype of the DLST gene is associated with Alztheimer‘s disease without a change of amino acid residues in the DLST protein: through a decrease in the expression of the truncated gene product MIRTD and the function of MIRTD. It remains unclear how much MIRTD actually contributes to the pathogenesis of Alzheimer‘s disease
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INHIBITORS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
coenzyme Q0
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formation of coenzyme Q0-dihydrolipoamide adducts in the E2 components of the alpha-ketoglutarate complex (i.e. dihydrolipoamide succinyltransferase) partially explains the cellular toxicity of coenzyme Q0
SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
SOURCE
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peripheral blood cells
Manually annotated by BRENDA team
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the DLST gene has two gene-products, one of which, gene product MIRTD, mediates the molecular assembly of the cytochrome c oxidase complex whose defect has been a candidate of the causes of Alzheimers disease. Since levels of MIRTD mRNA in the brains of Alzheimers disease patients are significantly low, a decrease in MIRTD could affect energy production
Manually annotated by BRENDA team
LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY hide
GeneOntology No.
LITERATURE
SOURCE
MOLECULAR WEIGHT
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
50000
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x * 50000, SDS-PAGE
SUBUNITS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
?
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x * 50000, SDS-PAGE
Purification/COMMENTARY
ORGANISM
UNIPROT
LITERATURE
Cloned/COMMENTARY
ORGANISM
UNIPROT
LITERATURE
isolation of the gene and pseudogene for the dihydrolipoamide succinyltransferase component of the human 2-oxoglutarate dehydrogenase complex from a human genomic DNA library, cloning vector lambda EMBL3 subcloned into plasmid vector pUC18, gene is located on chromosome 14 at q24.2-q24.3, pseudogene is located on chromosome at p31
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APPLICATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
medicine