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Literature summary for 2.3.1.61 extracted from

  • Kessler, M.; Berger, I.M.; Just, S.; Rottbauer, W.
    Loss of dihydrolipoyl succinyltransferase (DLST) leads to reduced resting heart rate in the zebrafish (2015), Basic Res. Cardiol., 110, 14.
    View publication on PubMedView publication on EuropePMC
Search for more literature for 2.3.1.61

Cloned(Commentary)

Cloned (Comment) Organism
gene zdlst, DNA and amino acid sequence determination and analysis, genetic fine-mapping, and quantitative real-time PCR enzyme expression analysis Danio rerio

Protein Variants

Protein Variants Comment Organism
additional information generation of enzyme knockout mutants, the zebrafish mutant schneckentempo, phenotype ste, exhibits bradycardia. The ste mutation is a guanine to adenine nucleotide transition in the splice donor site of intron 5 of the zdlst gene, ENSDARG00000014230 Danio rerio

Localization

Localization Comment Organism GeneOntology No. Textmining
mitochondrion the enzyme localizes to the mitochondrial matrix Danio rerio 5739
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Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
succinyl-CoA + enzyme N6-(dihydrolipoyl)lysine Danio rerio
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 CoA + enzyme N6-(S-succinyldihydrolipoyl)lysine
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 ?

Organism

Organism UniProt Comment Textmining
Danio rerio
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 gene zdlst
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Source Tissue

Source Tissue Comment Organism Textmining
heart
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 Danio rerio
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Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
succinyl-CoA + enzyme N6-(dihydrolipoyl)lysine
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 Danio rerio CoA + enzyme N6-(S-succinyldihydrolipoyl)lysine
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 ?

Synonyms

Synonyms Comment Organism
dihydrolipoyl succinyltransferase
-
 Danio rerio
DLST
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 Danio rerio

General Information

General Information Comment Organism
malfunction loss of dihydrolipoyl succinyltransferase leads to reduced resting heart rate in the zebrafish, enzyme deficiency causes the embryonic lethal, recessive zebrafish mutant schneckentempo ste phenotype. Homozygous ste mutants exhibit a severely reduced resting heart rate with normal atrio-ventricular conduction and contractile function. External electrical pacing reveals that defective excitation generation in cardiac pacemaker cells underlies bradycardia in ste-/- mutants. ATP levels are significantly diminished in ste-/- mutant embryos Danio rerio
metabolism dihydrolipoyl succinyltransferase catalyzes the citric acid cycle within the 2-oxoglutarate dehydrogenase complex Danio rerio
physiological function the mitochondrial enzyme dihydrolipoyl succinyltransferase is an essential player in the citric acid cycle that warrants proper ATP production. The enzyme plays an essential role in the modulation of the vertebrate heart rate by controlling ATP production in the heart Danio rerio