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Literature summary for 1.2.1.104 extracted from

  • Kitano, A.; Endo, F.; Kuroda, Y.; Aso, S.; Kawasaki, T.; Matsuda, I.
    Biochemical nature of pyruvate dehydrogenase complex in the patient with primary lactic acidaemia (1989), J. Inherit. Metab. Dis., 12, 379-385 .
    View publication on PubMed
Search for more literature for 1.2.1.104

Application

Application Comment Organism
medicine in a patient with primary lactic acidaemia, about 30% residual pyruvate dehydrogenase activity is observed, while the Km value is similiar to control. The enzyme deficiency is likely to be quantitative rather than qualitative. The patient developed severe metabolic acidosis at 8 months, accompanied by elevation of serum lactate and pyruvate. The lactate and pyruvate concentrations are also increased in cerevbrospinal fluid Homo sapiens

KM Value [mM]

KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
0.02
-
 pyruvate patient with primary lactic acidaemia, pH 8.0, temperature not specified in the publication Homo sapiens
0.058
-
 pyruvate healthy control, pH 8.0, temperature not specified in the publication Homo sapiens

Organism

Organism UniProt Comment Textmining
Homo sapiens P08559 and Q86SW4 P08559 i.e. E1 component subunit alpha, Q86SW4 i.e. dihydrolipoamide acetyltransferase component
-

Source Tissue

Source Tissue Comment Organism Textmining
muscle
-
 Homo sapiens
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
pyruvate + CoA + NAD+
-
 Homo sapiens acetyl-CoA + CO2 + NADH
-
 ?

Synonyms

Synonyms Comment Organism
dihydrolipoamide acetyltransferase
-
 Homo sapiens
DLST
-
 Homo sapiens
PdhA1
-
 Homo sapiens