CARNITINE THERAPY AND METABOLISM IN THE DISORDERS OF PROPIONYL-COA METABOLISM STUDIED USING H-1-NMR SPECTROSCOPY

被引:12
作者
DAVIES, SEC
ILES, RA
STACEY, TE
DESOUSA, C
CHALMERS, RA
机构
[1] UNIV LONDON LONDON HOSP,COLL MED,MED UNIT,CELLULAR MECHANISMS RES GRP,LONDON E1 1BB,ENGLAND
[2] MRC,CLIN RES CTR,PERINATAL & CHILD HLTH SECT,HARROW HA1 3UJ,MIDDX,ENGLAND
关键词
CARNITINE; INHERITED METABOLIC DISORDER; ORGANIC ACIDURIA; PROPIONIC ACIDEMIA; METHYLMALONIC ACIDURIA; NMR SPECTROSCOPY;
D O I
10.1016/0009-8981(91)90237-7
中图分类号
R446 [实验室诊断]; R-33 [实验医学、医学实验];
学科分类号
1001 ;
摘要
H-1-NMR spectroscopy has been used to study metabolic perturbations in patients with disorders of propionyl-CoA metabolism during the administration of oral and intravenous L-carnitine. The administration of L-carnitine either in the form of a challenge or as a therapeutic measure resulted in an increased excretion of propionylcarnitine, consistent with the removal of accumulated intramitochondrial propionyl-CoA esters. Additionally, during the therapeutic administration of L-carnitine excretion of acetylcarnitine occurred, coincident with an improvement in clinical condition and confirming the intracellular propionyl-CoA depletion. An additional benefit from the formation of acylcarnitines may be an accompanying intracellular alkalinisation.
引用
收藏
页码:263 / 277
页数:15
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