title: A method for quantitative measurement of mitochondrial creatine kinase in human skeletal muscle.
abstract: 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 (greater than 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).
authors.affiliation: Institute of Paediatrics, University Hospital Nijmegen, The Netherlands.
authors.forename: J
authors.initials: J
authors.lastname: Smeitink
authors.forename: R
authors.initials: R
authors.lastname: Wevers
authors.forename: J
authors.initials: J
authors.lastname: Hulshof
authors.forename: W
authors.initials: W
authors.lastname: Ruitenbeek
authors.forename: T
authors.initials: T
authors.lastname: van Lith
authors.forename: R
authors.initials: R
authors.lastname: Sengers
authors.forename: F
authors.initials: F
authors.lastname: Trijbels
authors.forename: C
authors.initials: C
authors.lastname: Korenke
authors.forename: T
authors.initials: T
authors.lastname: Wallimann
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D002846:Chromatography, Affinity; D003402:Creatine Kinase / Q000032:analysis*; D004588:Electrophoresis, Cellulose Acetate; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D008931:Mitochondria, Muscle / Q000201:enzymology*; D009468:Neuromuscular Diseases / Q000201:enzymology; D012016:Reference Values
chemical_list: D003402:Creatine Kinase