Plasma lysine concentration and availability of 2-ketoglutarate in liver mitochondria.

Abstract

Defects of lysine metabolism are rare, but hyperlysinemia is a concomitant of many inborn errors of metabolism, including urea cycle abnormalities, pyruvate carboxylase deficiency and L-2-hydroxyglutaric aciduria. We have hypothesized that mitochondrial lysine degradation is regulated by bioavailability of 2-oxoglutarate in the same compartment, and our studies in physiologic fluid derived from patients with the above described disorders supports our hypothsis. Our data further suggest that patients with isolated L-2-hydroxyglutaric aciduria may have a defect in 2-ketoglutarate metabolism. The current report summarizes our studies.

• Authors: P Kamoun, V Richard, D Rabier, J M Saudubray
• Journal: Journal of inherited metabolic disease (J Inherit Metab Dis) Vol. 25 Issue 1 Pg. 1-6 (Feb 2002) ISSN: 0141-8955 [Print] United States
• PMID: 11999975 (Publication Type: Journal Article)

Chemical References

• Ketoglutaric Acids
• Glutamine
• Glutamic Acid
• Lysine
• Alanine

Topics

• Alanine (blood)
• Biological Availability
• Glutamic Acid (blood)
• Glutamine (blood)
• Humans
• Infant
• Ketoglutaric Acids (metabolism, therapeutic use)
• Lysine (blood)
• Mitochondria, Liver (metabolism)