All issues > Volume 38(9); 1995

Original Article

J Korean Pediatr Soc. 1995;38(9):1167-1175. Published online September 15, 1995.

Measurement of Malonate Concentration using Malonyl-CoA Synthetase and its Relationship with Disease

Seung-Kyoo SK Han¹, Jeung-Kyu JK Kim¹, Seung-Ryeoul SR Paik², Chung-Soon CS Chang², Yu-San YS Kim³

¹Department of Pediatrics, College of Medicine, InHa University, Inchon, Korea
²Department of Biochemisty, College of Medicine, InHa University, Inchon, Korea
³Department of Biochemistry, College of Science, Yonsei University, Seoul. Korea

Abstract

Purpose
: Malonate is an effective competitive inhibitor of succinate dehydrogenase isolated from brain and other organs. It has been shown that malonate level is proportional to the growth of fetal rat brain. However, little is known about its biological effects on central nervous system and other organs. In this communication. we have measured malonate levels in neonates and patients with aseptic meninitis and determined their relationship with diseases.
Methods
: one hundred twenty five neonates and 25 children aged 18 months to 9 years admitted to InHa University Hospital from March 1993 to February 1994 had been analyzed in this study. The malonate concentrations in urine and cerebrospinal fluid were determined with malonyl-CoA synthetase from Rhizobiurn trifolii.
Results
: 1) The reference range of malonate concentration in urine during neonatal period was 0.04 to 0.15 mM with a median of 0.04 mM, which was within 95% confidence interval. Its distnbution showed an extremely right skewed shape. 2) From retrospective analysis of the neonates who showed significantly increased malonate concentrations in urine, it was found that sepsis, pathologic jaundice, or congenital anomalies such as ileal atresia and cleft lip and palate have been related to the level of malonate. 3) There was no significant difference of malonate concentrations between preterm and full-term neonates. 4) The mean cerebrospinal fluid concentrations of malonate, drawn from children with aseptic meningitis, was 0.080.02mM.
Conclusion
: The method introduced in this study using malonyl-CoA synthetase is more rapid and sensitive than any other methods available to date. Therefore, it can be used to evaluate and diagnose some diseases such as inborn error of metabolism. We believe, however, that further mass-survey and case-control studies on the diseases which exhibited significantly increased malonate concentrations are necessary to confirm the relationship.

Keywords :Malonate, Malonyl-CoA synthetase, Relationship with diseases