L-CARNITINE ENHANCES EXCRETION OF PROPIONYL COENZYME-A AS PROPIONYLCARNITINE IN PROPIONIC ACIDEMIA

被引:126
作者
ROE, CR
MILLINGTON, DS
MALTBY, DA
BOHAN, TP
HOPPEL, CL
机构
[1] CASE WESTERN RESERVE UNIV, VET ADM MED CTR, SCH MED, DEPT PHARMACOL, CLEVELAND, OH 44160 USA
[2] CASE WESTERN RESERVE UNIV, VET ADM MED CTR, SCH MED, DEPT MED, CLEVELAND, OH 44160 USA
关键词
D O I
10.1172/JCI111387
中图分类号
R-3 [医学研究方法]; R3 [基础医学];
学科分类号
1001 ;
摘要
引用
收藏
页码:1785 / 1788
页数:4
相关论文
共 17 条
[1]  
ALLEN RJ, 1982, LANCET, V2, P500
[2]   FAST ATOM BOMBARDMENT OF SOLIDS (FAB) - A NEW ION-SOURCE FOR MASS-SPECTROMETRY [J].
BARBER, M ;
BORDOLI, RS ;
SEDGWICK, RD ;
TYLER, AN .
JOURNAL OF THE CHEMICAL SOCIETY-CHEMICAL COMMUNICATIONS, 1981, (07) :325-327
[3]   PROPIONYLCARNITINE PHYSIOLOGICAL VARIATIONS IN VIVO [J].
BOHMER, T ;
BREMER, J .
BIOCHIMICA ET BIOPHYSICA ACTA, 1968, 152 (03) :559-&
[4]   RELATIONSHIP BETWEEN ACID-SOLUBLE CARNITINE AND COENZYME-A POOLS INVIVO [J].
BRASS, EP ;
HOPPEL, CL .
BIOCHEMICAL JOURNAL, 1980, 190 (03) :495-504
[5]  
BRASS EP, 1978, J BIOL CHEM, V253, P2688
[6]   INHIBITION BY PROPIONYL-COENZYME-A OF N-ACETYLGLUTAMATE SYNTHETASE IN RAT-LIVER MITOCHONDRIA - POSSIBLE EXPLANATION FOR HYPER-AMMONEMIA IN PROPIONIC AND METHYLMALONIC ACIDEMIA [J].
COUDE, FX ;
SWEETMAN, L ;
NYHAN, WL .
JOURNAL OF CLINICAL INVESTIGATION, 1979, 64 (06) :1544-1551
[7]   C-6-C-10-DICARBOXYLIC ACIDURIA - INVESTIGATIONS OF A PATIENT WITH RIBOFLAVIN RESPONSIVE MULTIPLE ACYL-COA DEHYDROGENATION DEFECTS [J].
GREGERSEN, N ;
WINTZENSEN, H ;
KOLVRAA, S ;
CHRISTENSEN, E ;
CHRISTENSEN, MF ;
BRANDT, NJ ;
RASMUSSEN, K .
PEDIATRIC RESEARCH, 1982, 16 (10) :861-868
[8]   THE SPECIFIC-INHIBITION OF THE PYRUVATE-DEHYDROGENASE COMPLEX FROM PIG-KIDNEY BY PROPIONYL-COA AND ISOVALERYL-COA [J].
GREGERSEN, N .
BIOCHEMICAL MEDICINE, 1981, 26 (01) :20-27
[9]  
HAYASAKA K, 1982, PEDIATR RES, V16, P5
[10]   URINARY-EXCRETION OF ACETYLCARNITINE DURING HUMAN DIABETIC AND FASTING KETOSIS [J].
HOPPEL, CL ;
GENUTH, SM .
AMERICAN JOURNAL OF PHYSIOLOGY, 1982, 243 (02) :E168-E172