CEREBRAL LACTIC-ACIDOSIS - DEFECTS IN PYRUVATE METABOLISM WITH PROFOUND BRAIN-DAMAGE AND MINIMAL SYSTEMIC ACIDOSIS

被引:93
作者
BROWN, GK [1 ]
HAAN, EA [1 ]
KIRBY, DM [1 ]
SCHOLEM, RD [1 ]
WRAITH, JE [1 ]
ROGERS, JG [1 ]
DANKS, DM [1 ]
机构
[1] UNIV MELBOURNE,DEPT PAEDIAT,PARKVILLE,VIC 3052,AUSTRALIA
关键词
D O I
10.1007/BF00442603
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
引用
收藏
页码:10 / 14
页数:5
相关论文
共 13 条
[1]   PYRUVATE-CARBOXYLASE AND PHOSPHOENOLPYRUVATE CARBOXYKINASE ACTIVITY IN LEUKOCYTES AND FIBROBLASTS FROM A PATIENT WITH PYRUVATE-CARBOXYLASE DEFICIENCY [J].
ATKIN, BM ;
UTTER, MF ;
WEINBERG, MB .
PEDIATRIC RESEARCH, 1979, 13 (01) :38-43
[2]   MALONYL COENZYME-A DECARBOXYLASE DEFICIENCY [J].
BROWN, GK ;
SCHOLEM, RD ;
BANKIER, A ;
DANKS, DM .
JOURNAL OF INHERITED METABOLIC DISEASE, 1984, 7 (01) :21-26
[3]   NEUROPATHOLOGY IN CEREBRAL LACTIC-ACIDOSIS [J].
CHOW, CW ;
ANDERSON, RM ;
KENNY, GCT .
ACTA NEUROPATHOLOGICA, 1987, 74 (04) :393-396
[4]   ACTIVITY OF PYRUVATE-DEHYDROGENASE IN RAT-BRAIN DURING POSTNATAL-DEVELOPMENT [J].
CREMER, JE ;
TEAL, HM .
FEBS LETTERS, 1974, 39 (01) :17-20
[5]  
King T.E., 1967, METHODS ENZYMOL, V10, P275, DOI [10.1016/0076-6879(67)10055-4, DOI 10.1016/0076-6879(67)10055-4]
[6]  
PRICK M, 1981, NEUROLOGY, V31, P398
[7]   LACTIC ACIDEMIA [J].
ROBINSON, BH ;
SHERWOOD, WG .
JOURNAL OF INHERITED METABOLIC DISEASE, 1984, 7 :69-73
[8]   THE GENETIC-HETEROGENEITY OF LACTIC-ACIDOSIS - OCCURRENCE OF RECOGNIZABLE INBORN-ERRORS OF METABOLISM IN A PEDIATRIC POPULATION WITH LACTIC-ACIDOSIS [J].
ROBINSON, BH ;
TAYLOR, J ;
SHERWOOD, WG .
PEDIATRIC RESEARCH, 1980, 14 (08) :956-962
[9]   A STUDY OF SOME OXIDATIVE ENZYMES OF BAKERS YEAST [J].
SMITH, L .
ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS, 1954, 50 (02) :285-298
[10]   DISORDERS OF THE PYRUVATE-DEHYDROGENASE COMPLEX [J].
STANSBIE, D ;
WALLACE, SJ ;
MARSAC, C .
JOURNAL OF INHERITED METABOLIC DISEASE, 1986, 9 (02) :105-119