Inborn errors of the Krebs cycle: a group of unusual mitochondrial diseases in human, Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease, vol.1361, issue.2, pp.1361185-197, 1997. ,
DOI : 10.1016/S0925-4439(97)00035-5
Tricarboxylic acid cycle dysfunction as a cause of human diseases and tumor formation, AJP: Cell Physiology, vol.291, issue.6, pp.1114-1120, 2006. ,
DOI : 10.1152/ajpcell.00216.2006
The Mitochondrion in Health and Diseases, 1992. ,
Energy deficiency in the failing heart: Linking increased reactive oxygen species and disruption of oxidative phosphorylation rate, Biochimica et Biophysica Acta (BBA) - Bioenergetics, vol.1757, issue.5-6, pp.5-6543, 2006. ,
DOI : 10.1016/j.bbabio.2006.03.008
Proteomic analysis of mitochondrial proteins in cardiomyocytes from chronic stressed rat, PROTEOMICS, vol.4, issue.10, pp.3167-3176, 2004. ,
DOI : 10.1002/pmic.200300845
Aconitase and mitochondrial iron???sulphur protein deficiency in Friedreich ataxia, Nature Genetics, vol.12, issue.2, pp.215-217, 1997. ,
DOI : 10.1016/0009-8981(94)90055-8
Respiratory chain defects: what do we know for sure about their consequences in vivo?, Biochimica et Biophysica Acta (BBA) - Bioenergetics, vol.1659, issue.2-3, pp.172-177, 2004. ,
DOI : 10.1016/j.bbabio.2004.07.002
Accumulation of Krebs cycle intermediates and over-expression of HIF1alpha in tumours which result from germline FH and SDH mutations, Hum Mol Genet, issue.15, pp.142231-2239, 2005. ,
The Constant Proportion Enzyme Group Concept in the Selection of Reference Enzymes in Metabolism, Ciba Found Symp, vol.60, pp.231-244, 1979. ,
DOI : 10.1002/9780470720561.ch13
Further characterization of the Krebs tricarboxylic acid cycle metabolon, J Biol Chem, vol.262, issue.4, pp.1786-1790, 1987. ,
Supramolecular organization of tricarboxylic acid cycle enzymes, Biosystems, vol.22, issue.2, pp.91-102, 1989. ,
DOI : 10.1016/0303-2647(89)90038-5
A novel branched-chain amino acid metabolon. Proteinprotein interactions in a supramolecular complex, J Biol Chem, issue.16, pp.28211893-11903, 2007. ,
Aspartate aminotransferase in synaptic and nonsynaptic mitochondria: Differential effect of compounds that influence transient hetero-enzyme complex (metabolon) formation, Neurochemistry International, vol.48, issue.6-7, pp.6-7629, 2006. ,
DOI : 10.1016/j.neuint.2005.11.018
Assessment of the mitochondrial respiratory chain, The Lancet, vol.338, issue.8758, p.33860, 1991. ,
DOI : 10.1016/0140-6736(91)90057-V
Biochemical and molecular investigations in respiratory chain deficiencies, Clinica Chimica Acta, vol.228, issue.1, pp.35-51, 1994. ,
DOI : 10.1016/0009-8981(94)90055-8
Regulation of Metabolic Processes in Mitochondria, 1966. ,
Mitochondrial activities in human cultured skin fibroblasts contaminated by Mycoplasma hyorhinis, BMC Biochemistry, vol.4, issue.1, p.15, 2003. ,
DOI : 10.1186/1471-2091-4-15
URL : https://hal.archives-ouvertes.fr/inserm-00114072
Mutation of the fumarase gene in two siblings with progressive encephalopathy and fumarase deficiency., Journal of Clinical Investigation, vol.93, issue.6, pp.932514-2518, 1994. ,
DOI : 10.1172/JCI117261
Mutation of a nuclear succinate dehydrogenase gene results in mitochondrial respiratory chain deficiency, Nature Genetics, vol.267, issue.2, pp.144-149, 1995. ,
DOI : 10.1016/0378-1119(93)90238-X
The citric acid cycle and the Szent-Gy??rgyi cycle in pigeon breast muscle, Biochemical Journal, vol.34, issue.5, pp.775-779, 1940. ,
DOI : 10.1042/bj0340775
Comparable and specific proportions in the mitochondrial enzyme activity pattern, Biochemical and Biophysical Research Communications, vol.7, issue.6, pp.425-429, 1962. ,
DOI : 10.1016/0006-291X(62)90328-5
Diagnostic Assays for Respiratory Syncytial Virus Disease, The Pediatric Infectious Disease Journal, vol.26, issue.Supplement, pp.36-40, 2007. ,
DOI : 10.1097/INF.0b013e318157da6f
Biochemical Assays of Respiratory Chain Complex Activity, Methods Cell Biol, vol.80, pp.93-119, 2007. ,
DOI : 10.1016/S0091-679X(06)80004-X
Prerequisites and strategies for prenatal diagnosis of respiratory chain deficiency in chorionic villi, Journal of Inherited Metabolic Disease, vol.26, issue.7, pp.647-658, 2003. ,
DOI : 10.1023/B:BOLI.0000005605.57420.b4
Mutations in SDHD, a Mitochondrial Complex II Gene, in Hereditary Paraganglioma, Science, vol.287, issue.5454, pp.287848-851, 2000. ,
DOI : 10.1126/science.287.5454.848
Germline mutations in FH predispose to dominantly inherited uterine fibroids, skin leiomyomata and papillary renal cell cancer, Nature Genetics, vol.30, issue.4, pp.406-410, 2002. ,
DOI : 10.1038/ng849
A novel mutation in the dihydrolipoamide dehydrogenase E3 subunit gene (DLD) resulting in an atypical form of ??-ketoglutarate dehydrogenase deficiency, Human Mutation, vol.25, issue.3, pp.323-324, 2005. ,
DOI : 10.1002/humu.9319
Fate and expression of the deleted mitochondrial DNA differ between human heteroplasmic skin fibroblast and Epstein-Barr virus-transformed lymphocyte cultures, J Biol Chem, issue.26, pp.26819369-19376, 1993. ,
Assay of mitochondrial respiratory chain complex I in human lymphocytes and cultured skin fibroblasts, Biochemical and Biophysical Research Communications, vol.301, issue.1, pp.222-224, 2003. ,
DOI : 10.1016/S0006-291X(02)03016-4
A rapid and sensitive method for the quantitation of microgram quantities of protein utilizing the principle of protein-dye binding, Analytical Biochemistry, vol.72, issue.1-2, pp.248-254, 1976. ,
DOI : 10.1016/0003-2697(76)90527-3
Neuro-oncology: Isocitrate dehydrogenase mutations in low-grade gliomas, Nature Reviews Neurology, vol.116, issue.6, pp.303-304, 2009. ,
DOI : 10.1038/nrneurol.2009.57
Deficiency of the ADPforming succinyl-CoA synthase activity is associated with encephalomyopathy and mitochondrial DNA depletion, Am J Hum Genet, issue.6, pp.761081-1086, 2005. ,
Succinate links TCA cycle dysfunction to oncogenesis by inhibiting HIF-?? prolyl hydroxylase, Cancer Cell, vol.7, issue.1, pp.77-85, 2005. ,
DOI : 10.1016/j.ccr.2004.11.022
URL : http://doi.org/10.1016/j.ccr.2004.11.022