ArticleThe Biochemical journal1998
Biochemical parameters for the diagnosis of mitochondrial respiratory chain deficiency in humans, and their lack of age-related changes.
Article in The Biochemical journal, 1998. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers.
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Who cites it
20 citing papers in PubMed, 98 citations in OpenAlex.
- Effects of Exercise and Aging on Skeletal Muscle.Cold Spring Harbor perspectives in medicine · 2018Review
- Mitochondrial structure and function are not different between nonfailing donor and end-stage failing human hearts.FASEB journal : official publication of the Federation of American Societies for Experimental Biology · 2016Article
- A novel method for determining human ex vivo submaximal skeletal muscle mitochondrial function.The Journal of physiology · 2015Article
- Mitochondrial and skeletal muscle health with advancing age.Molecular and cellular endocrinology · 2013Review
- Skeletal muscle aging and the mitochondrion.Trends in endocrinology and metabolism: TEM · 2013Review
- Age-related changes in oxidative capacity differ between locomotory muscles and are associated with physical activity behavior.Applied physiology, nutrition, and metabolism = Physiologie appliquee, nutrition et metabolisme · 2012Article
- Biomarkers for mitochondrial respiratory chain disorders.Journal of inherited metabolic disease · 2011Review
- Regulation of skeletal muscle mitochondrial function: genes to proteins.Acta physiologica (Oxford, England) · 2010Review
- Article
- Evaluation of the mitochondrial respiratory chain and oxidative phosphorylation system using polarography and spectrophotometric enzyme assays.Current protocols in human genetics · 2009Article
- The coordination of nuclear and mitochondrial communication during aging and calorie restriction.Ageing research reviews · 2009Review
- Loss of Aif function causes cell death in the mouse embryo, but the temporal progression of patterning is normal.Proceedings of the National Academy of Sciences of the United States of America · 2006Article
- Decline in skeletal muscle mitochondrial function with aging in humans.Proceedings of the National Academy of Sciences of the United States of America · 2005Article
- Mitochondrial activities in human cultured skin fibroblasts contaminated by Mycoplasma hyorhinis.BMC biochemistry · 2003Article
- Human skeletal muscle mitochondrial metabolism in youth and senescence: no signs of functional changes in ATP formation and mitochondrial oxidative capacity.Pflugers Archiv : European journal of physiology · 2003Article
- Mitochondrial oxidative phosphorylation: pitfalls and tips in measuring and interpreting enzyme activities.Journal of inherited metabolic disease · 2003Review
- Electron transport chain defects in heart failure.Heart failure reviews · 2002Review
- Impaired redox status and cytochrome c oxidase deficiency in patients with polymyalgia rheumatica.Annals of the rheumatic diseases · 2001Article
- Dramatic improvement in mitochondrial cardiomyopathy following treatment with idebenone.Journal of inherited metabolic disease · 2001Article
- Mutations of the SCO1 gene in mitochondrial cytochrome c oxidase deficiency with neonatal-onset hepatic failure and encephalopathy.American journal of human genetics · 2000Article
Corrections and comments
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Authors and funding
8 authors at 4 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
It is now widely acknowledged that a large number of human diseases originate from respiratory-chain dysfunctions. Because the molecular bases of these diseases are still poorly known, a biochemical approach has to be used in the screening procedures for the diagnoses of these conditions. Assessment of respiratory-chain function in human samples faces several problems: (i) the small size of available samples, (ii) the determination of discriminating parameters, and (iii) the interfering factors, such as age and physical activity. The present study focuses on isolated mitochondria prepared from a minute amount (100-200 mg) of skeletal-muscle biopsies from 201 patients between 0 and 65 years. Whereas 42 patients presented an isolated complex (C)I, CII, CIII or CIV deficiency, no respiratory-chain dysfunction or indirect evidence for a mitochondrial disorder could be attested in 159 of these patients. In this reference group, there was little correlation between enzyme activities and age, whatever the age class considered, 0-3 or 0-65 years of age. However, a confident handling of data points was largely hampered by the marked scattering of enzyme activities measured in the reference population. Activity ratios between the various respiratory-chain complexes presenting a much reduced scattering may be considered as diagnostic tools. As to the effect of age, no correlation with any of the enzyme-activity ratios could be shown. Use of age-matched controls for the diagnosis of respiratory-chain disorders may therefore be avoided, enzyme-activity ratios being highly discriminating and age-independent parameters.
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