DEFICIENCY OF DIHYDROLIPOYL DEHYDROGENASE (A COMPONENT OF PYRUVATE AND ALPHA-KETOGLUTARATE DEHYDROGENASE COMPLEXES) - CAUSE OF CONGENITAL CHRONIC LACTIC-ACIDOSIS IN INFANCY

被引:128
作者
ROBINSON, BH
TAYLOR, J
SHERWOOD, WG
机构
[1] UNIV TORONTO,HOSP SICK CHILDREN,DEPT PAEDIAT,TORONTO M5G 1X8,ONTARIO,CANADA
[2] UNIV TORONTO,DEPT BIOCHEM,TORONTO,ONTARIO,CANADA
[3] HOSP SICK CHILDREN,RES INST,TORONTO M5G 1X8,ONTARIO,CANADA
关键词
D O I
10.1203/00006450-197712000-00006
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
引用
收藏
页码:1198 / 1202
页数:5
相关论文
共 36 条
[1]   ALPHA-KETO ACID DEHYDROGENASE COMPLEXES .16. STUDIES ON SUBUNIT STRUCTURE OF PYRUVATE DEHYDROGENASE COMPLEXES FROM BOVINE KIDNEY AND HEART [J].
BARRERA, CR ;
REED, LJ ;
LINN, TC ;
MUNK, P ;
NAMIHIRA, G ;
ELEY, MH ;
HAMILTON, L .
ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS, 1972, 148 (02) :343-+
[2]  
BERGMEYER HU, 1965, METHOD ENZYMAT AN, P324
[3]  
BLASS J P, 1970, Journal of Clinical Investigation, V49, P423, DOI 10.1172/JCI106251
[4]   CLINICAL STUDIES OF A PATIENT WITH PYRUVATE DECARBOXYLASE DEFICIENCY [J].
BLASS, JP ;
KARK, RAP ;
ENGEL, WK .
ARCHIVES OF NEUROLOGY, 1971, 25 (05) :449-&
[5]  
BLASS JP, 1971, LANCET, V1, P1302
[6]  
BUCHER T, 1965, METHOD ENZYMAT AN, P253
[7]   SENSITIVITY TO CARBOHYDRATE IN A PATIENT WITH FAMILIAL INTERMITTENT LACTIC-ACIDOSIS AND PYRUVATE-DEHYDROGENASE DEFICIENCY [J].
CEDERBAUM, SD ;
BLASS, JP ;
MINKOFF, N ;
BROWN, WJ ;
COTTON, ME ;
HARRIS, SH .
PEDIATRIC RESEARCH, 1976, 10 (08) :713-720
[8]   ACTIVITIES OF PYRUVATE CARBOXYLASE, PHOSPHOENOLPYRUVATE CARBOXYLASE AND FRUCTOSE DIPHOSPHATASE IN MUSCLES FROM VERTEBRATES AND INVERTEBRATES [J].
CRABTREE, B ;
NEWSHOLME, EA ;
HIGGINS, SJ .
BIOCHEMICAL JOURNAL, 1972, 130 (02) :391-+
[9]   ACTIVITY OF PYRUVATE-DEHYDROGENASE IN RAT-BRAIN DURING POSTNATAL-DEVELOPMENT [J].
CREMER, JE ;
TEAL, HM .
FEBS LETTERS, 1974, 39 (01) :17-20
[10]  
FARMER TW, 1973, NEUROLOGY, V23, P429