DISPROPORTIONATE DEFICIENCY OF IRON-SULFUR CLUSTERS AND SUBUNITS OF COMPLEX-I IN MITOCHONDRIAL ENCEPHALOMYOPATHY

被引:23
作者
ICHIKI, T
TANAKA, M
KOBAYASHI, M
SUGIYAMA, N
SUZUKI, H
NISHIKIMI, M
OHNISHI, T
NONAKA, I
WADA, Y
OZAWA, T
机构
[1] NAGOYA UNIV,FAC MED,DEPT BIOMED CHEM,65 TSURUMA CHO,SHOWA KU,NAGOYA,AICHI 466,JAPAN
[2] NAGOYA CITY UNIV,SCH MED,DEPT PEDIAT,NAGOYA,AICHI 467,JAPAN
[3] UNIV PENN,DEPT BIOCHEM & BIOPHYS,PHILADELPHIA,PA 19104
[4] NATL CTR NEUROL & PSYCHIAT,KODAIRA,TOKYO 187,JAPAN
关键词
D O I
10.1203/00006450-198902000-00023
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
引用
收藏
页码:194 / 201
页数:8
相关论文
共 42 条
[1]   SEQUENCE AND ORGANIZATION OF THE HUMAN MITOCHONDRIAL GENOME [J].
ANDERSON, S ;
BANKIER, AT ;
BARRELL, BG ;
DEBRUIJN, MHL ;
COULSON, AR ;
DROUIN, J ;
EPERON, IC ;
NIERLICH, DP ;
ROE, BA ;
SANGER, F ;
SCHREIER, PH ;
SMITH, AJH ;
STADEN, R ;
YOUNG, IG .
NATURE, 1981, 290 (5806) :457-465
[2]   CHARACTERIZATION OF IRON-SULFUR PROTEIN OF MITOCHONDRIAL OUTER MEMBRANE PARTIALLY PURIFIED FROM BEEF KIDNEY CORTEX [J].
BACKSTROM, D ;
LORUSSO, M ;
ANDERSON, K ;
EHRENBERG, A .
BIOCHIMICA ET BIOPHYSICA ACTA, 1978, 502 (02) :276-288
[3]   PYRUVATE OXIDATION IN RAT AND HUMAN SKELETAL-MUSCLE MITOCHONDRIA [J].
BOOKELMAN, H ;
TRIJBELS, JMF ;
SENGERS, RCA ;
JANSSEN, AJM ;
VEERKAMP, JH ;
STADHOUDERS, AM .
BIOCHEMICAL MEDICINE, 1978, 20 (03) :395-403
[4]   URF6, LAST UNIDENTIFIED READING FRAME OF HUMAN MTDNA, CODES FOR AN NADH DEHYDROGENASE SUBUNIT [J].
CHOMYN, A ;
CLEETER, MWJ ;
RAGAN, CI ;
RILEY, M ;
DOOLITTLE, RF ;
ATTARDI, G .
SCIENCE, 1986, 234 (4776) :614-618
[5]  
COOPERSTEIN SJ, 1951, J BIOL CHEM, V189, P665
[6]   MITOCHONDRIAL MYOPATHIES [J].
DIMAURO, S ;
BONILLA, E ;
ZEVIANI, M ;
NAKAGAWA, M ;
DEVIVO, DC .
ANNALS OF NEUROLOGY, 1985, 17 (06) :521-538
[7]   MITOCHONDRIAL CYTOPATHY - A MULTISYSTEM DISORDER WITH RAGGED RED FIBERS ON MUSCLE BIOPSY [J].
EGGER, J ;
LAKE, BD ;
WILSON, J .
ARCHIVES OF DISEASE IN CHILDHOOD, 1981, 56 (10) :741-752
[8]  
Galante Y M, 1978, Methods Enzymol, V53, P15
[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