DEFICIENCY OF THE REDUCED NICOTINAMIDE ADENINE-DINUCLEOTIDE DEHYDROGENASE COMPONENT OF COMPLEX-I OF MITOCHONDRIAL ELECTRON-TRANSPORT - FATAL INFANTILE LACTIC-ACIDOSIS AND HYPERMETABOLISM WITH SKELETAL-CARDIAC MYOPATHY AND ENCEPHALOPATH

被引:151
作者
HOPPEL, CL
KERR, DS
DAHMS, B
ROESSMANN, U
机构
[1] UNIV HOSP CLEVELAND, CLEVELAND, OH 44106 USA
[2] RAINBOW BABIES & CHILDRENS HOSP, DEPT MED, CLEVELAND, OH 44106 USA
[3] RAINBOW BABIES & CHILDRENS HOSP, DEPT PEDIAT, CLEVELAND, OH 44106 USA
[4] RAINBOW BABIES & CHILDRENS HOSP, DEPT PHARMACOL, CLEVELAND, OH 44106 USA
[5] RAINBOW BABIES & CHILDRENS HOSP, DEPT PATHOL, CLEVELAND, OH 44106 USA
[6] RAINBOW BABIES & CHILDRENS HOSP, DEPT BIOCHEM, CLEVELAND, OH 44106 USA
关键词
D O I
10.1172/JCI113066
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:71 / 77
页数:7
相关论文
共 32 条
[1]   STUDIES ON ELECTRON TRANSFER SYSTEM .54. ISOLATION OF UNIT OF ELECTRON TRANSFER [J].
BLAIR, PV ;
GREEN, DE ;
ODA, T .
BIOCHEMISTRY, 1963, 2 (04) :756-&
[2]  
BRASS EP, 1978, J BIOL CHEM, V253, P2688
[3]  
BUSCH HFM, 1981, MITOCHONDRIA MUSCULA, P207
[4]  
CHANCE B, 1955, J BIOL CHEM, V217, P409
[5]   MYOPATHIES DUE TO ENZYME DEFICIENCIES [J].
CORNELIO, F ;
DIDONATO, S .
JOURNAL OF NEUROLOGY, 1985, 232 (06) :329-340
[6]   MITOCHONDRIAL MYOPATHIES [J].
DIMAURO, S ;
BONILLA, E ;
ZEVIANI, M ;
NAKAGAWA, M ;
DEVIVO, DC .
ANNALS OF NEUROLOGY, 1985, 17 (06) :521-538
[7]  
Estabrook R.W., 1967, METHOD ENZYMOL, V10, P41, DOI DOI 10.1016/0076-6879(67)10010-4
[8]  
FOMON SJ, 1974, INFANT NUTRITION, P23
[9]  
Hatefi Y, 1978, Methods Enzymol, V53, P11
[10]   THE MITOCHONDRIAL ELECTRON-TRANSPORT AND OXIDATIVE-PHOSPHORYLATION SYSTEM [J].
HATEFI, Y .
ANNUAL REVIEW OF BIOCHEMISTRY, 1985, 54 :1015-1069