SECONDARILY REDUCED CYTOCHROME-C-OXIDASE ACTIVITY IN VARIOUS NEUROMUSCULAR DISORDERS

被引:6
作者
OHTAKI, E [1 ]
机构
[1] KURUME UNIV,SCH MED,DEPT PEDIAT & CHILD HLTH,KURUME,FUKUOKA 830,JAPAN
关键词
Cytochrome c oxidase; denervated muscle; mitochondrial myopathy; neuromuscular diseases; rat muscle;
D O I
10.1016/S0387-7604(12)80315-9
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Decreased cytochrome c oxidase (CCO) activity was found in various neuromuscular diseases, including infantile spinal muscular atrophy (SMA) and Fukuyama type congenital muscular dystrophy (FCMD), that was thought to result from a secondarily induced mitochondrial defect. To determine whether or not the enzyme activities in the mitochondrial electron transport system can be secondarily changed, we measured the enzyme activities in denervated rat muscles. The CCO activity decreased progressively to 38–47% of the control value in 3 weeks after denervation, the NADH-cytochrome c reductase and succinate-cytochrome c reductase activities remaining unchanged, suggesting that the CCO activity may be easily reduced secondarily in various disease conditions. © 1990, All rights reserved.
引用
收藏
页码:326 / 333
页数:8
相关论文
共 50 条
[21]   SEGMENTAL CYTOCHROME-C-OXIDASE DEFICIENCY IN CPEO - TEASED MUSCLE-FIBER ANALYSIS [J].
MATSUOKA, T ;
GOTO, YI ;
HASEGAWA, H ;
NONAKA, I .
MUSCLE & NERVE, 1992, 15 (02) :209-213
[22]   INTERACTION OF CARBONYL CYANIDE 3-CHLOROPHENYLHYDRAZONE WITH CYTOCHROME-C-OXIDASE [J].
BONA, M ;
ANTALIK, M ;
GAZOVA, Z ;
KUCHAR, A ;
DADAK, V ;
PODHRADSKY, D .
GENERAL PHYSIOLOGY AND BIOPHYSICS, 1993, 12 (06) :533-542
[23]   ALTERED KINETICS OF CYTOCHROME-C-OXIDASE IN A PATIENT WITH SEVERE MITOCHONDRIAL ENCEPHALOMYOPATHY [J].
NIJTMANS, LGJ ;
BARTH, PG ;
LINCKE, CR ;
VANGALEN, MJM ;
ZWART, R ;
KLEMENT, P ;
BOLHUIS, PA ;
RUITENBEEK, W ;
WANDERS, RJA ;
VANDENBOGERT, C .
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE, 1995, 1270 (2-3) :193-201
[24]   DIBUCAINE INTERACTS DIFFERENTLY WITH MEMBRANE AND PROTEIN IN CYTOCHROME-C-OXIDASE SYSTEMS [J].
HE, J ;
NICHOLLS, P .
BIOCHEMISTRY AND CELL BIOLOGY-BIOCHIMIE ET BIOLOGIE CELLULAIRE, 1993, 71 (1-2) :14-21
[25]   A NEW SPECTRAL INTERMEDIATE IN CYANIDE BINDING WITH THE OXIDIZED CYTOCHROME-C-OXIDASE [J].
BERKA, V ;
VYGODINA, T ;
MUSATOV, A ;
NICHOLLS, P ;
KONSTANTINOV, AA .
FEBS LETTERS, 1993, 315 (03) :237-241
[26]   PROTON UPTAKE BY CYTOCHROME-C-OXIDASE ON REDUCTION AND ON LIGAND-BINDING [J].
MITCHELL, R ;
RICH, PR .
BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS, 1994, 1186 (1-2) :19-26
[27]   MITOCHONDRIAL ENCEPHALOMYOPATHIES AND CYTOCHROME-C-OXIDASE DEFICIENCY - MUSCLE CULTURE STUDY [J].
NONAKA, I ;
KOGA, Y ;
KIKUCHI, A ;
GOTO, Y .
ACTA NEUROPATHOLOGICA, 1991, 82 (04) :286-294
[28]   THE PATHWAY OF CO BINDING TO CYTOCHROME-C-OXIDASE - CAN THE GATEWAY BE CLOSED [J].
HILL, BC .
FEBS LETTERS, 1994, 354 (03) :284-288
[29]   CHARACTERIZATION OF FAST AND SLOW FORMS OF BOVINE HEART CYTOCHROME-C-OXIDASE [J].
MOODY, AJ ;
COOPER, CE ;
RICH, PR .
BIOCHIMICA ET BIOPHYSICA ACTA, 1991, 1059 (02) :189-207
[30]   ISOFORMS OF CYTOCHROME-C-OXIDASE IN TISSUES AND CELL-LINES OF THE MOUSE [J].
VANDENBOGERT, C ;
DEKKER, HL ;
CORNELISSEN, JC ;
VANKUILENBURG, ABP ;
BOLHUIS, PA ;
MUIJSERS, AO .
BIOCHIMICA ET BIOPHYSICA ACTA, 1992, 1099 (02) :118-122