A METHOD FOR QUANTITATIVE MEASUREMENT OF MITOCHONDRIAL CREATINE-KINASE IN HUMAN SKELETAL-MUSCLE

被引:20
作者
SMEITINK, J
WEVERS, R
HULSHOF, J
RUITENBEEK, W
VONLITH, T
SENGERS, R
TRIJBELS, F
KORENKE, C
WALLIMANN, T
机构
[1] UNIV HOSP NIJMEGEN, INST PAEDIAT, NIJMEGEN, NETHERLANDS
[2] UNIV HOSP NIJMEGEN, INST NEUROL, NIJMEGEN, NETHERLANDS
[3] SWISS FED INST TECHNOL, INST CELL BIOL, CH-8092 ZURICH, SWITZERLAND
关键词
ENZYME EXTRACTION; CK ISOENZYMES; MITOCHONDRIAL MYOPATHY;
D O I
10.1177/000456329202900213
中图分类号
R446 [实验室诊断]; R-33 [实验医学、医学实验];
学科分类号
1001 ;
摘要
Defects in the mitochondrial energy generating system in patients with a mitochondrial myopathy are known to be localized in various enzyme complexes involved in energy production. Such a defect may exist at the level of mitochondrial creatine kinase (Mi-CK). On that account we have developed a method for measurement of the enzyme activity in human skeletal muscle biopsy material (> 10 mg). Interfering creatine kinase isoenzymes are removed by anion exchange and affinity chromatography. The activity of Mi-CK in reference skeletal muscle homogenates amounts to 240 +/- 88 mU/mg protein (30 +/- 8.0 mU/mg wet weight).
引用
收藏
页码:196 / 201
页数:6
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