CLINICAL AND BIOCHEMICAL IMPLICATIONS OF PYRUVATE-CARBOXYLASE DEFICIENCY

被引:106
作者
DEVIVO, DC
HAYMOND, MW
LECKIE, MP
BUSSMANN, YL
MCDOUGAL, DB
PAGLIARA, AS
机构
[1] WASHINGTON UNIV, SCH MED, DEPT NEUROL, ST LOUIS, MO 63130 USA
[2] WASHINGTON UNIV, SCH MED, DEPT NEUROSURG NEUROL, ST LOUIS, MO 63130 USA
[3] WASHINGTON UNIV, SCH MED, DEPT PHARMACOL, ST LOUIS, MO 63130 USA
[4] WASHINGTON UNIV, SCH MED, EDWARD MALLINCKRODT DEPT PEDIAT, ST LOUIS, MO 63130 USA
[5] ST LOUIS CHILDRENS HOSP, DIV NEUROL, ST LOUIS, MO 63110 USA
[6] ST LOUIS CHILDRENS HOSP, DIV METAB, ST LOUIS, MO 63110 USA
关键词
D O I
10.1210/jcem-45-6-1281
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
引用
收藏
页码:1281 / 1296
页数:16
相关论文
共 63 条
[1]  
ADAM PAJ, 1969, J BIOL CHEM, V244, P6444
[2]   IMMUNOCHEMICAL STUDIES WITH SOLUBLE AND MITOCHRONDRIAL PYRUVATE CARBOXYLASE ACTIVITIES FROM RAT TISSUES [J].
BALLARD, FJ ;
HANSON, RW ;
RESHEF, L .
BIOCHEMICAL JOURNAL, 1970, 119 (04) :735-&
[3]  
Barritt G. J., 1976, GLUCONEOGENESIS ITS, P3
[4]   COMPARISON OF COMPETITIVE PROTEIN BINDING RADIOASSAY OF CORTISOL TO DOUBLE ISOTOPE DILUTION AND PORTER SILBER METHODS [J].
BEITINS, IZ ;
SHAW, MH ;
KOWARSKI, A ;
MIGEON, CJ .
STEROIDS, 1970, 15 (06) :765-&
[5]  
BLASS JP, 1976, LANCET, V1, P1237
[6]  
BRUNETTE MG, 1972, PEDIATRICS, V50, P702
[7]   HORMONE-FUEL INTERRELATIONSHIPS DURING FASTING [J].
CAHILL, GF ;
HERRERA, MG ;
MORGAN, AP ;
SOELDNER, JS ;
STEINKE, J ;
LEVY, PL ;
REICHARD, GA ;
KIPINS, DM .
JOURNAL OF CLINICAL INVESTIGATION, 1966, 45 (11) :1751-+
[8]   ACTIVITY OF PYRUVATE-DEHYDROGENASE IN RAT-BRAIN DURING POSTNATAL-DEVELOPMENT [J].
CREMER, JE ;
TEAL, HM .
FEBS LETTERS, 1974, 39 (01) :17-20
[9]   PYRUVATE-CARBOXYLASE IN HUMAN LIVER - APPARENT LOSS OF A COMPONENT OF CATALYTIC ACTIVITY IN A FORM OF LACTIC ACIDOSIS WITH HYPOGLYCEMIA [J].
DELVIN, E ;
SCRIVER, CR ;
NEAL, JL .
BIOCHEMICAL MEDICINE, 1974, 10 (02) :97-106
[10]  
DEVIVO DC, 1976, J NEUROCHEM, V26, P635, DOI 10.1111/j.1471-4159.1976.tb01526.x