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Literature summary extracted from

  • Szabo, E.; Mizsei, R.; Wilk, P.; Zambo, Z.; Torocsik, B.; Weiss, M.S.; Adam-Vizi, V.; Ambrus, A.
    Crystal structures of the disease-causing D444V mutant and the relevant wild type human dihydrolipoamide dehydrogenase (2018), Free Radic. Biol. Med., 124, 214-220 .
    View publication on PubMed

Cloned(Commentary)

EC Number Cloned (Comment) Organism
1.8.1.4 expressed in Escherichia coli Homo sapiens

Crystallization (Commentary)

EC Number Crystallization (Comment) Organism
1.8.1.4 sitting drop vapor diffusion method, using 0.1 M Bis-Tris pH 7.45, 0.2 M MgCl2, 25% (w/v) PEG 3350 for the wild type enzyme and 1.6 M NaH2PO4/K2HPO4 pH 8.1 plus 2.5 M K2HPO4 for mutant enzyme D444V Homo sapiens

Protein Variants

EC Number Protein Variants Comment Organism
1.8.1.4 D444V the mutation causes microcephaly, blindness, deafness, mild hypertrophic cardiomyopathy, and metabolic acidosis. The substitution leads to compromised enzyme activity (15% of the control) Homo sapiens

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
1.8.1.4 protein N6-(dihydrolipoyl)lysine + NAD+ Homo sapiens
-
 protein N6-(lipoyl)lysine + NADH + H+
-
 ?

Organism

EC Number Organism UniProt Comment Textmining
1.8.1.4 Homo sapiens
-
-
-

Purification (Commentary)

EC Number Purification (Comment) Organism
1.8.1.4
-
 Homo sapiens

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
1.8.1.4 dihydrolipoamide + NAD+
-
 Homo sapiens lipoamide + NADH + H+
-
 ?
1.8.1.4 additional information the enzyme catalyzes the reoxidation of the covalently bound reduced lipoic acid cofactor of the E2 subunits of all the mitochondrial alpha-keto acid dehydrogenase multienzyme complexes including the alpha-ketoglutarate dehydrogenase complex, the pyruvate dehydrogenase complex, and the branched-chain alpha-keto acid dehydrogenase complex Homo sapiens ?
-
-
1.8.1.4 protein N6-(dihydrolipoyl)lysine + NAD+
-
 Homo sapiens protein N6-(lipoyl)lysine + NADH + H+
-
 ?

Subunits

EC Number Subunits Comment Organism
1.8.1.4 homodimer 2 * 50000, SDS-PAGE Homo sapiens

Synonyms

EC Number Synonyms Comment Organism
1.8.1.4 dihydrolipoamide dehydrogenase
-
 Homo sapiens
1.8.1.4 E3
-
 Homo sapiens
1.8.1.4 LADH
-
 Homo sapiens

Cofactor

EC Number Cofactor Comment Organism Structure
1.8.1.4 FAD
-
 Homo sapiens
1.8.1.4 NAD+
-
 Homo sapiens