Journal of the Korean Pediatric Society 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 Han1, Jeung-Kyu Kim1, Seung-Ryeoul Paik2, Chung-Soon Chang2, Yu-San 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
Malonyl-CoA Synthetase를 이용한 Malonate 농도 측정 및 질병과의 상관성에 관한 연구 (제 1보)
한승규1, 김정규1, 백승렬2, 장정순2, 김유삼3
1인하대학교 의과대학 소아과학교실
2인하대학교 의과대학 생화학교실
3연세대학교 이과대학 생화학과
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.
Key Words: Malonate, Malonyl-CoA synthetase, Relationship with diseases


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