Pierre Rustin

Q: What Schools Are Affiliated With Pierre Rustin

Pierre Rustin is affiliated with the following schools: Université Paris Cité, Paris Diderot University, Karolinska Institute, Sorbonne University

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Pierre Rustin

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#7498

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#10719

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#5852

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Philosophy

Pierre Rustin's Degrees

PhD Biochemistry Université Paris Cité
Masters Molecular Biology Université Paris Cité

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Pierre Rustin's Published Works

Number of citations in a given year to any of this author's works

Total number of citations to an author for the works they published in a given year. This highlights publication of the most important work(s) by the author

Published Works

Mitochondrial transcription factor A is necessary for mtDNA maintance and embryogenesis in mice (1998) (1479)
Biochemical and molecular investigations in respiratory chain deficiencies. (1994) (1201)
Aconitase and mitochondrial iron–sulphur protein deficiency in Friedreich ataxia (1997) (968)
Accumulation of Krebs cycle intermediates and over-expression of HIF1alpha in tumours which result from germline FH and SDH mutations. (2005) (845)
Mitochondrial transcription factor A regulates mtDNA copy number in mammals. (2004) (778)
Mouse models for Friedreich ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits (2001) (685)
Mutation of a nuclear succinate dehydrogenase gene results in mitochondrial respiratory chain deficiency (1995) (684)
Persistent mitochondrial dysfunction and perinatal exposure to antiretroviral nucleoside analogues (1999) (657)
SDH mutations establish a hypermethylator phenotype in paraganglioma. (2013) (617)
SDHA is a tumor suppressor gene causing paraganglioma. (2010) (617)
AIF deficiency compromises oxidative phosphorylation (2004) (597)
Defects in succinate dehydrogenase in gastrointestinal stromal tumors lacking KIT and PDGFRA mutations (2010) (559)
Mutations in the SDHB gene are associated with extra-adrenal and/or malignant phaeochromocytomas. (2003) (513)
Pearson's marrow-pancreas syndrome. A multisystem mitochondrial disorder in infancy. (1990) (440)
Clinical and molecular genetics of patients with the Carney–Stratakis syndrome and germline mutations of the genes coding for the succinate dehydrogenase subunits SDHB, SDHC, and SDHD (2008) (438)
Dilated cardiomyopathy and atrioventricular conduction blocks induced by heart-specific inactivation of mitochondrial DNA gene expression (1999) (432)
Spermidine and resveratrol induce autophagy by distinct pathways converging on the acetylproteome (2011) (421)
Targeted Deletion of AIF Decreases Mitochondrial Oxidative Phosphorylation and Protects from Obesity and Diabetes (2007) (386)
The R22X mutation of the SDHD gene in hereditary paraganglioma abolishes the enzymatic activity of complex II in the mitochondrial respiratory chain and activates the hypoxia pathway. (2001) (357)
Imbalanced OPA1 processing and mitochondrial fragmentation cause heart failure in mice (2015) (351)
Mutations of the SCO1 gene in mitochondrial cytochrome c oxidase deficiency with neonatal-onset hepatic failure and encephalopathy. (2000) (347)
Quinone-responsive multiple respiratory-chain dysfunction due to widespread coenzyme Q10 deficiency (2000) (346)
Effect of idebenone on cardiomyopathy in Friedreich's ataxia: a preliminary study (1999) (338)
Germline mutations in FH confer predisposition to malignant pheochromocytomas and paragangliomas. (2014) (330)
A mutant mitochondrial respiratory chain assembly protein causes complex III deficiency in patients with tubulopathy, encephalopathy and liver failure (2001) (297)
Persistent mitochondrial dysfunction in HIV-1-exposed but uninfected infants: clinical screening in a large prospective cohort (2003) (290)
Control of Mitochondrial Membrane Permeabilization by Adenine Nucleotide Translocator Interacting with HIV-1 Viral Protein R and Bcl-2 (2001) (289)
A mutation in the human heme A:farnesyltransferase gene (COX10 ) causes cytochrome c oxidase deficiency. (2000) (282)
Increased in vivo apoptosis in cells lacking mitochondrial DNA gene expression (2001) (252)
Large-scale deletion and point mutations of the nuclear NDUFV1 and NDUFS1 genes in mitochondrial complex I deficiency. (2001) (247)
Clinical spectrum and diagnosis of mitochondrial disorders. (2001) (237)
Mitochondria are physiologically maintained at close to 50 °C (2017) (235)
Idebenone and reduced cardiac hypertrophy in Friedreich's ataxia (2002) (231)
The Warburg Effect Is Genetically Determined in Inherited Pheochromocytomas (2009) (225)
Succinate dehydrogenase and human diseases: new insights into a well-known enzyme (2002) (223)
Functional consequences of a SDHB gene mutation in an apparently sporadic pheochromocytoma. (2002) (206)
The human cytochrome c oxidase assembly factors SCO1 and SCO2 have regulatory roles in the maintenance of cellular copper homeostasis. (2007) (203)
Muscle-Specific Loss of Apoptosis-Inducing Factor Leads to Mitochondrial Dysfunction, Skeletal Muscle Atrophy, and Dilated Cardiomyopathy (2005) (194)
Cytochrome oxidase in health and disease. (2002) (190)
Disabled early recruitment of antioxidant defenses in Friedreich's ataxia. (2001) (188)
Mutant NDUFS3 subunit of mitochondrial complex I causes Leigh syndrome (2004) (186)
Spectrum of mitochondrial DNA rearrangements in the Pearson marrow-pancreas syndrome. (1995) (183)
Genetic modification of survival in tissue-specific knockout mice with mitochondrial cardiomyopathy. (2000) (183)
Friedreich Ataxia Mouse Models with Progressive Cerebellar and Sensory Ataxia Reveal Autophagic Neurodegeneration in Dorsal Root Ganglia (2004) (181)
S6 kinase deletion suppresses muscle growth adaptations to nutrient availability by activating AMP kinase. (2007) (178)
Impaired Nuclear Nrf2 Translocation Undermines the Oxidative Stress Response in Friedreich Ataxia (2009) (172)
Maternally inherited duplication of the mitochondrial genome in a syndrome of proximal tubulopathy, diabetes mellitus, and cerebellar ataxia. (1992) (170)
Mutant NDUFV2 subunit of mitochondrial complex I causes early onset hypertrophic cardiomyopathy and encephalopathy (2003) (169)
Coenzyme Q(10) and idebenone in the therapy of respiratory chain diseases: rationale and comparative benefits. (2002) (162)
Late-Onset Corticohippocampal Neurodepletion Attributable to Catastrophic Failure of Oxidative Phosphorylation in MILON Mice (2001) (160)
Stabilization of Hypoxia-inducible Factor-1α Protein in Hypoxia Occurs Independently of Mitochondrial Reactive Oxygen Species Production* (2010) (157)
Mitochondrial succinate is instrumental for HIF1alpha nuclear translocation in SDHA-mutant fibroblasts under normoxic conditions. (2005) (156)
Expression of the Ciona intestinalis alternative oxidase (AOX) in Drosophila complements defects in mitochondrial oxidative phosphorylation. (2009) (155)
Deletion of mitochondrial DNA in a case of early-onset diabetes mellitus, optic atrophy, and deafness (Wolfram syndrome, MIM 222300). (1993) (154)
Superoxide-induced massive apoptosis in cultured skin fibroblasts harboring the neurogenic ataxia retinitis pigmentosa (NARP) mutation in the ATPase-6 gene of the mitochondrial DNA. (2001) (151)
Impaired mitochondrial glutamate transport in autosomal recessive neonatal myoclonic epilepsy. (2005) (151)
Cytochrome c Oxidase Subassemblies in Fibroblast Cultures from Patients Carrying Mutations in COX10, SCO1, or SURF1* (2004) (148)
Mutation of the fumarase gene in two siblings with progressive encephalopathy and fumarase deficiency. (1994) (148)
Compound heterozygous mutations in the flavoprotein gene of the respiratory chain complex II in a patient with Leigh syndrome (2000) (143)
Interaction between AIF and CHCHD4 Regulates Respiratory Chain Biogenesis. (2015) (141)
The mitochondrial DNA G13513A MELAS mutation in the NADH dehydrogenase 5 gene is a frequent cause of Leigh-like syndrome with isolated complex I deficiency (2003) (139)
Succinate dehydrogenase (SDH) D subunit (SDHD) inactivation in a growth-hormone-producing pituitary tumor: a new association for SDH? (2012) (138)
Idebenone treatment in Friedreich patients: One-year-long randomized placebo-controlled trial (2004) (138)
A deletion in the human QP-C gene causes a complex III deficiency resulting in hypoglycaemia and lactic acidosis (2003) (137)
The central role of malate in plant metabolism (1984) (134)
Reference charts for respiratory chain activities in human tissues. (1994) (130)
A case of mitochondrial encephalomyopathy associated with a muscle coenzyme Q10 deficiency (1998) (129)
Inborn errors of the Krebs cycle: a group of unusual mitochondrial diseases in human. (1997) (126)
Inborn errors of complex II--unusual human mitochondrial diseases. (2002) (126)
Clinical presentation of mitochondrial disorders in childhood (1996) (125)
Idebenone delays the onset of cardiac functional alteration without correction of Fe-S enzymes deficit in a mouse model for Friedreich ataxia. (2004) (125)
BH3 mimetics activate multiple pro-autophagic pathways (2011) (124)
Expression of the yeast NADH dehydrogenase Ndi1 in Drosophila confers increased lifespan independently of dietary restriction (2010) (124)
Allotopic expression of a mitochondrial alternative oxidase confers cyanide resistance to human cell respiration (2006) (121)
PGC-1α is Dispensable for Exercise-Induced Mitochondrial Biogenesis in Skeletal Muscle (2012) (120)
Antenatal manifestations of mitochondrial respiratory chain deficiency. (2003) (119)
Branched chain amino acids induce apoptosis in neural cells without mitochondrial membrane depolarization or cytochrome c release: implications for neurological impairment associated with maple syrup urine disease. (2000) (119)
Recurrent de novo mitochondrial DNA mutations in respiratory chain deficiency (2003) (118)
Mitochondria, from cell death to proliferation (2002) (117)
Mitochondrial cytochrome c oxidase deficiency. (2016) (111)
The spectrum of systemic involvement in adults presenting with renal lesion and mitochondrial tRNA(Leu) gene mutation. (2003) (111)
Novel FH mutations in families with hereditary leiomyomatosis and renal cell cancer (HLRCC) and patients with isolated type 2 papillary renal cell carcinoma (2011) (107)
Alternative Oxidase Expression in the Mouse Enables Bypassing Cytochrome c Oxidase Blockade and Limits Mitochondrial ROS Overproduction (2013) (103)
Tricarboxylic acid cycle dysfunction as a cause of human diseases and tumor formation. (2006) (103)
Respiratory-chain diseases related to complex III deficiency. (2009) (101)
A mitochondrial cytochrome b mutation but no mutations of nuclearly encoded subunits in ubiquinol cytochrome c reductase (complex III) deficiency (1999) (100)
Loss of Aif function causes cell death in the mouse embryo, but the temporal progression of patterning is normal. (2006) (100)
Disconnecting mitochondrial content from respiratory chain capacity in PGC-1-deficient skeletal muscle. (2013) (97)
Clinical aspects of mitochondrial disorders (1992) (96)
The optimized allotopic expression of ND1 or ND4 genes restores respiratory chain complex I activity in fibroblasts harboring mutations in these genes. (2008) (96)
Fate and expression of the deleted mitochondrial DNA differ between human heteroplasmic skin fibroblast and Epstein-Barr virus-transformed lymphocyte cultures. (1993) (96)
Clinical presentations and laboratory investigations in respiratory chain deficiency (1996) (96)
Cytopathic effects of the cytomegalovirus-encoded apoptosis inhibitory protein vMIA (2006) (96)
Assessment of the mitochondrial respiratory chain (1991) (94)
Biochemical parameters for the diagnosis of mitochondrial respiratory chain deficiency in humans, and their lack of age-related changes. (1998) (94)
Co-amplification of nuclear pseudogenes and assessment of heteroplasmy of mitochondrial DNA mutations. (1998) (94)
Absence of relationship between the level of electron transport chain activities and aging in human skeletal muscle. (1996) (92)
Heart Hypertrophy and Function Are Improved by Idebenone in Friedreich's Ataxia (2002) (90)
Expression of the alternative oxidase complements cytochrome c oxidase deficiency in human cells (2009) (89)
Three spectrophotometric assays for the measurement of the five respiratory chain complexes in minuscule biological samples. (2006) (88)
A critical approach to the therapy of mitochondrial respiratory chain and oxidative phosphorylation diseases. (2009) (87)
Neonatal and delayed-onset liver involvement in disorders of oxidative phosphorylation. (1997) (86)
Hereditary Paraganglioma/Pheochromocytoma and Inherited Succinate Dehydrogenase Deficiency (2005) (86)
Allotopic mRNA localization to the mitochondrial surface rescues respiratory chain defects in fibroblasts harboring mitochondrial DNA mutations affecting complex I or v subunits. (2007) (84)
Malate Oxidation in Plant Mitochondria via Malic Enzyme and the Cyanide-insensitive Electron Transport Pathway. (1980) (84)
A high rate (20%-30%) of parental consanguinity in cytochrome-oxidase deficiency. (1998) (82)
Hair and skin disorders as signs of mitochondrial disease. (1999) (81)
Aging is associated with increased lipid peroxidation in human hearts, but not with mitochondrial respiratory chain enzyme defects. (2000) (79)
An improved spectrophotometric assay of pyruvate dehydrogenase in lactate dehydrogenase contaminated mitochondrial preparations from human skeletal muscle. (1995) (79)
Succinate dehydrogenase deficiency in human (2005) (78)
Cytochrome c oxidase assay in minute amounts of human skeletal muscle using single wavelength spectrophotometers (1998) (77)
Alpha-ketoglutarate dehydrogenase deficiency presenting as congenital lactic acidosis. (1992) (76)
The Variability of the Harlequin Mouse Phenotype Resembles that of Human Mitochondrial-Complex I-Deficiency Syndromes (2008) (76)
Endomyocardial biopsies for early detection of mitochondrial disorders in hypertrophic cardiomyopathies. (1994) (75)
Mitochondrial DNA rearrangements with onset as chronic diarrhea with villous atrophy. (1994) (73)
Deficiency in complex II of the respiratory chain, presenting as a leukodystrophy in two sisters with leigh syndrome (1992) (71)
Unsuspected task for an old team: succinate, fumarate and other Krebs cycle acids in metabolic remodeling. (2014) (71)
Acute and chronic mitochondrial respiratory chain deficiency differentially regulate lysosomal biogenesis (2017) (71)
A novel mutation in the dihydrolipoamide dehydrogenase E3 subunit gene (DLD) resulting in an atypical form of α‐ketoglutarate dehydrogenase deficiency (2005) (70)
Friedreich's ataxia: the vicious circle hypothesis revisited (2011) (67)
Real-time flow cytometry analysis of permeability transition in isolated mitochondria. (2004) (66)
Quinone analogues regulate mitochondrial substrate competitive oxidation. (2004) (65)
Mitochondrial dysfunction following perinatal exposure to nucleoside analogues (2006) (64)
Compound heterozygous mutations in the flavoprotein gene of the respiratory chain complex II in a patient with Leigh syndrome. (2000) (64)
A simple and accurate spectrophotometric assay for phosphoenolpyruvate carboxylase activity. (1988) (64)
The measurement of the rotenone-sensitive NADH cytochrome c reductase activity in mitochondria isolated from minute amount of human skeletal muscle. (1990) (63)
Deferiprone targets aconitase: Implication for Friedreich's ataxia treatment (2008) (63)
Assay of mitochondrial respiratory chain complex I in human lymphocytes and cultured skin fibroblasts. (2003) (63)
Genotyping microsatellite DNA markers at putative disease loci in inbred/multiplex families with respiratory chain complex I deficiency allows rapid identification of a novel nonsense mutation (IVS1nt −1) in the NDUFS4 gene in Leigh syndrome (2003) (63)
In Vitro and In Vivo Modulation of Alternative Splicing by the Biguanide Metformin (2015) (63)
HIV-1 Tat protein directly induces mitochondrial membrane permeabilization and inactivates cytochrome c oxidase (2012) (59)
Fluxes of Nicotinamide Adenine Dinucleotides through Mitochondrial Membranes in Human Cultured Cells* (1996) (58)
RXRα overexpression in cardiomyocytes causes dilated cardiomyopathy but fails to rescue myocardial hypoplasia in RXRα-null fetuses (2000) (57)
Mitochondrial complex I deficiency of nuclear origin I. Structural genes. (2012) (57)
Isolation and characterization of mitochondria from human B lymphoblastoid cell lines. (1992) (56)
Glutathione precursors replenish decreased glutathione pool in cystinotic cell lines. (2004) (56)
Fatal lactic acidosis and liver steatosis associated with didanosine and stavudine treatment: a respiratory chain dysfunction? (2000) (56)
Deletion of mitochondrial DNA in patient with chronic tubulointerstitial nephritis. (1995) (54)
Long-term Follow-up of Neonatal Mitochondrial Cytopathies: A Study of 57 Patients (2005) (53)
Mutations in CYC1, encoding cytochrome c1 subunit of respiratory chain complex III, cause insulin-responsive hyperglycemia. (2013) (53)
Expression of alternative oxidase in Drosophila ameliorates diverse phenotypes due to cytochrome oxidase deficiency (2013) (53)
Mitochondrial respiratory chain defect: a new etiology for neonatal cholestasis and early liver insufficiency. (1995) (52)
Engineering the alternative oxidase gene to better understand and counteract mitochondrial defects: state of the art and perspectives (2014) (52)
Neuroglobin involvement in respiratory chain function and retinal ganglion cell integrity. (2012) (52)
Mitochondrial biogenesis and development of respiratory chain enzymes in kidney cells: role of glucocorticoids. (1994) (51)
The neurogenic weakness, ataxia and retinitis pigmentosa (NARP) syndrome mtDNA mutation (T8993G) triggers muscle ATPase deficiency and hypocitrullinaemia (1999) (51)
Mitochondrial response to controlled nutrition in health and disease. (2011) (51)
Animal models for respiratory chain disease. (2001) (50)
In Vivo Detection of Succinate by Magnetic Resonance Spectroscopy as a Hallmark of SDHx Mutations in Paraganglioma (2015) (50)
Dramatic improvement in mitochondrial cardiomyopathy following treatment with idebenone (2001) (50)
Hepatic failure in disorders of oxidative phosphorylation with neonatal onset. (1991) (50)
Refractory anaemia and mitochondrial cytopathy in childhood (1994) (49)
Broad AOX expression in a genetically tractable mouse model does not disturb normal physiology (2017) (48)
Mitochondria are physiologically maintained at close to 50 degrees C (2018) (48)
Respiratory chain alternative enzymes as tools to better understand and counteract respiratory chain deficiencies in human cells and animals. (2009) (48)
Wide clinical variability among 13 new Cockayne syndrome cases confirmed by biochemical assays (2005) (47)
The environmental carcinogen benzo[a]pyrene induces a Warburg-like metabolic reprogramming dependent on NHE1 and associated with cell survival (2016) (47)
The molecular archaeology of a mitochondrial death effector: AIF in Drosophila (2008) (47)
SLC25A32 Mutations and Riboflavin-Responsive Exercise Intolerance. (2016) (47)
Use of Human Cancer Cell Lines Mitochondria to Explore the Mechanisms of BH3 Peptides and ABT-737-Induced Mitochondrial Membrane Permeabilization (2010) (47)
Supernumerary subunits NDUFA3, NDUFA5 and NDUFA12 are required for the formation of the extramembrane arm of human mitochondrial complex I (2014) (46)
Segregation of the G8993 mutant mitochondrial DNA through generations and embryonic tissues in a family at risk of Leigh syndrome. (1997) (46)
Mitochondrial complex I deficiency of nuclear origin II. Non-structural genes. (2012) (46)
Craniofacial anomalies and malformations in respiratory chain deficiency. (1996) (46)
Investigation of the role of SDHB inactivation in sporadic phaeochromocytoma and neuroblastoma (2004) (46)
Apoptosis-Inducing Factor Deficiency Induces Early Mitochondrial Degeneration in Brain Followed by Progressive Multifocal Neuropathology (2007) (44)
Glucose Modulates Respiratory Complex I Activity in Response to Acute Mitochondrial Dysfunction (2012) (44)
Clinical homogeneity of the Stüve-Wiedemann syndrome and overlap with the Schwartz-Jampel syndrome type 2. (1998) (44)
Targeting neonatal ischemic brain injury with a pentapeptide-based irreversible caspase inhibitor (2011) (44)
Heart transplantation in children with mitochondrial cardiomyopathy (2001) (43)
Molecular insights into Friedreich's ataxia and antioxidant-based therapies. (2002) (43)
Mitochondria transfection by oligonucleotides containing a signal peptide and vectorized by cationic liposomes. (2001) (43)
Tubulointerstitial nephritis and Fanconi syndrome in primary biliary cirrhosis. (2005) (42)
Downregulation of apoptosis-inducing factor in Harlequin mice induces progressive and severe optic atrophy which is durably prevented by AAV2-AIF1 gene therapy. (2012) (42)
Mutations in SURF1 are not specifically associated with Leigh syndrome (2001) (41)
Efficiency of metabolic screening in childhood cardiomyopathies. (1998) (40)
OXPHOS defects and mitochondrial DNA mutations in cardiomyopathy (1995) (40)
Cerebral white matter disease in children may be caused by mitochondrial respiratory chain deficiency. (2000) (40)
The gene responsible for Dyggve-Melchior-Clausen syndrome encodes a novel peripheral membrane protein dynamically associated with the Golgi apparatus. (2009) (40)
For debate: defective mitochondria, free radicals, cell death, aging-reality or myth-ochondria? (2000) (39)
QIL1 mutation causes MICOS disassembly and early onset fatal mitochondrial encephalopathy with liver disease (2016) (39)
Respiratory chain deficiency presenting as congenital nephrotic syndrome (2005) (38)
Genotypes and clinical phenotypes in children with cytochrome-c oxidase deficiency. (2003) (38)
Mutations in the Dimer Interface of Dihydrolipoamide Dehydrogenase Promote Site-specific Oxidative Damages in Yeast and Human Cells* (2011) (38)
Respiratory chain defects: what do we know for sure about their consequences in vivo? (2004) (37)
Deoxyguanosine kinase mutations and combined deficiencies of the mitochondrial respiratory chain in patients with hepatic involvement. (2005) (36)
Longevity-relevant regulation of autophagy at the level of the acetylproteome (2011) (35)
Valproate-induced hepatic failure in a case of cytochromec oxidase deficiency (1994) (35)
CHCHD2 accumulates in distressed mitochondria and facilitates oligomerization of CHCHD10 (2018) (35)
From Nf1 to Sdhb knockout: Successes and failures in the quest for animal models of pheochromocytoma (2016) (35)
Respiratory chain deficiency in Alpers syndrome. (2001) (35)
A brain-specific isoform of mitochondrial apoptosis-inducing factor: AIF2 (2010) (34)
Biochemical, genetic and immunoblot analyses of 17 patients with an isolated cytochrome c oxidase deficiency. (1999) (34)
KBP-cytoskeleton interactions underlie developmental anomalies in Goldberg-Shprintzen syndrome. (2013) (34)
Cells Lacking the Fumarase Tumor Suppressor Are Protected from Apoptosis through a Hypoxia-Inducible Factor-Independent, AMPK-Dependent Mechanism (2012) (34)
Quinone analogs prevent enzymes targeted in Friedreich ataxia from iron‐induced injury in vitro (1999) (34)
Mitochondria and diabetes mellitus: untangling a conflictive relationship? (2009) (34)
Mitochondrial oxidative phosphorylation: Pitfalls and tips in measuring and interpreting enzyme activities (2003) (32)
Respiratory chain defects may present only with hypoglycemia. (2005) (32)
Genetic background influences mitochondrial function: modeling mitochondrial disease for therapeutic development. (2010) (32)
Determination of enzyme activities for prenatal diagnosis of respiratory chain deficiency (2000) (31)
Targeted Vpr-derived peptides reach mitochondria to induce apoptosis of αVβ3-expressing endothelial cells (2007) (31)
Expression of the alternative oxidase mitigates beta-amyloid production and toxicity in model systems. (2016) (31)
Presentation of six cases of Stüve-Wiedemann syndrome (1998) (31)
The use of antioxidants in Friedreich’s ataxia treatment (2003) (30)
Rapid determination of tricarboxylic acid cycle enzyme activities in biological samples (2010) (30)
Coenzyme Q 10 Depletion is Comparatively Less Detrimental to Human Cultured Skin Fibroblasts than Respiratory Chain Complex Deficiencies (2002) (30)
The alternative oxidase, a tool for compensating cytochrome c oxidase deficiency in human cells. (2009) (30)
Clinical mitochondrial dysfunction in uninfected children born to HIV‐infected mothers following perinatal exposure to nucleoside analogues (2007) (29)
Pro-oxidant/antioxidant balance controls pancreatic β-cell differentiation through the ERK1/2 pathway (2014) (29)
Interaction between Mitochondrial Cytochromes and Linoleic Acid Hydroperoxide: POSSIBLE CONFUSION WITH LIPOXYGENASE AND ALTERNATIVE PATHWAY. (1982) (29)
Oxidative stress induces mitochondrial fragmentation in frataxin-deficient cells. (2012) (29)
Kinetic studies of the form of substrate bound by phosphoenolpyruvate carboxylase. (1988) (28)
Evolutionarily conserved susceptibility of the mitochondrial respiratory chain to SDHI pesticides and its consequence on the impact of SDHIs on human cultured cells (2019) (28)
Expression of respiratory chain deficiencies in human cultured cells (1993) (28)
Lipin1 deficiency causes sarcoplasmic reticulum stress and chaperone‐responsive myopathy (2018) (27)
Changes in oxidative properties of Kalanchoe blossfeldiana leaf mitochondria during development of Crassulacean acid metabolism (1985) (27)
Synthesis and preliminary biological evaluations of ionic and nonionic amphiphilic alpha-phenyl-N-tert-butylnitrone derivatives. (2003) (26)
Targeted Vpr-derived peptides reach mitochondria to induce apoptosis of alphaVbeta3-expressing endothelial cells. (2007) (26)
Cytochrome c oxidase deficiency presenting as recurrent neonatal myoglobinuria (1995) (26)
Cis-silencing of PIP5K1B evidenced in Friedreich's ataxia patient cells results in cytoskeleton anomalies. (2013) (26)
Identification of substrate and effector binding sites of phosphoenolpyruvate carboxylase from Crassula argentea. A possible role of phosphoenolpyruvate as substrate and activator. (1988) (26)
Genetic and biochemical intricacy shapes mitochondrial cytopathies (2016) (26)
Revisiting Pitfalls, Problems and Tentative Solutions for Assaying [General Articles] Mitochondrial Respiratory Chain Complex III in Human Samples (2004) (26)
Persistent hypocitrullinaemia as a marker for mtDNA NARP T 8993 G mutation? (1998) (25)
Mouse Studies to Shape Clinical Trials for Mitochondrial Diseases: High Fat Diet in Harlequin Mice (2011) (25)
Biochemical investigations and immunoblot analyses of two unrelated patients with an isolated deficiency in complex II of the mitochondrial respiratory chain. (1996) (24)
Involvement of lipid peroxy radicals in the cyanide-resistant electron transport pathway (1984) (24)
Mitochondrial respiratory chain dysfunction caused by coenzyme Q deficiency. (2004) (24)
Malate Metabolism in Leaf Mitochondria from the Crassulacean Acid Metabolism Plant Kalanchoë blossfeldiana Poelln. (1986) (23)
No mitochondrial cytochrome oxidase (COX) gene mutations in 18 cases of COX deficiency (1997) (23)
Translocator Protein-Mediated Stabilization of Mitochondrial Architecture during Inflammation Stress in Colonic Cells (2016) (23)
A role for fatty acid peroxy radicals in the cyanide-insensitive pathway of plant mitochondria? (1983) (23)
Uridine preserves the expression of respiratory enzyme deficiencies in cultured fibroblasts (1993) (22)
RXRalpha overexpression in cardiomyocytes causes dilated cardiomyopathy but fails to rescue myocardial hypoplasia in RXRalpha-null fetuses. (2000) (22)
Alternative respiratory chain enzymes: Therapeutic potential and possible pitfalls. (2019) (22)
Actinomycin D Targets NPM1c-Primed Mitochondria to Restore PML-Driven Senescence in AML Therapy (2021) (22)
The consequences of a mild respiratory chain deficiency on substrate competitive oxidation in human mitochondria. (1997) (22)
Effect of Lon protease knockdown on mitochondrial function in HeLa cells. (2014) (22)
Kidney involvement in mitochondrial disorders. (1995) (21)
Another observation with VATER association and a complex IV respiratory chain deficiency. (2006) (20)
The investigation of respiratory chain disorders in heart using endomyocardial biopsies (1993) (20)
Therapies in inborn errors of oxidative metabolism (2012) (20)
A cancer‐predisposing “hot spot” mutation of the fumarase gene creates a dominant negative protein (2008) (20)
Succinate-driven reverse electron transport in the respiratory chain of plant mitochondria. The effects of rotenone and adenylates in relation to malate and oxaloacetate metabolism. (1991) (19)
A kinetic study of the effects of phosphate and organic phosphates on the activity of phosphoenolpyruvate carboxylase from Crassula argentea. (1989) (19)
Malic enzyme activity and cyanide-insensitive electron transport in plant mitochondria. (1979) (19)
Oxidative interactions between fatty acid peroxy radicals and quinones: possible involvement in cyanide-resistant electron transport in plant mitochondria. (1983) (18)
ACO2 mutations: A novel phenotype associating severe optic atrophy and spastic paraplegia (2018) (18)
Decylubiquinol impedes mitochondrial respiratory chain complex I activity (2008) (18)
Cell complementation using Genebridge 4 human:rodent hybrids for physical mapping of novel mitochondrial respiratory chain deficiency genes. (2002) (18)
HIF1α and pancreatic β‐cell development (2012) (17)
Investigation of respiratory chain activity in human heart. (1993) (17)
Deletion of mitochondrial DNA in a case of early-onset diabetes mellitus, optic atrophy and deafness (DIDMOAD, Wolfram syndrome) (1993) (17)
Mitochondrial respiratory chain deficiencies expressing the enzymatic deficiency in the hepatic tissue: a study of 31 patients. (2006) (17)
Mitochondrial activities in human cultured skin fibroblasts contaminated by Mycoplasma hyorhinis (2003) (17)
Human cultured skin fibroblasts survive profound inherited ubiquinone depletion (2001) (16)
Mitochondrial Complex I Deficiency in Humans (2004) (16)
The NDUFB6 subunit of the mitochondrial respiratory chain complex I is required for electron transfer activity: a proof of principle study on stable and controlled RNA interference in human cell lines. (2011) (16)
Cell Metabolic Alterations due to Mcph1 Mutation in Microcephaly. (2020) (16)
Diiron centre mutations in Ciona intestinalis alternative oxidase abolish enzymatic activity and prevent rescue of cytochrome oxidase deficiency in flies (2015) (15)
Succinate detection using in vivo 1H-MR spectroscopy identifies germline and somatic SDHx mutations in paragangliomas (2019) (15)
Limitations in a frataxin knockdown cell model for Friedreich ataxia in a high-throughput drug screen (2009) (15)
Respiratory chain deficiency presenting as recurrent myoglobinuria in childhood. (1999) (13)
The influence of pH on substrate form specificity of phosphoenolpyruvate carboxylase purified from Crassula argentea. (1990) (13)
The mtDNA NARP mutation activates the actin-Nrf2 signaling of antioxidant defenses. (2008) (13)
Alteration of mitochondrial DNA and RNA level in human fibroblasts with impaired vitamin B12 coenzyme synthesis (1998) (12)
Prenatal diagnosis of respiratory chain deficiency by direct mutation screening (2001) (12)
Revisiting pitfalls, problems and tentative solutions for assaying mitochondrial respiratory chain complex III in human samples. (2004) (12)
Mitochondria Are Physiologically Maintained At Close To 50 C (2017) (12)
Pitfalls in Monitoring Mitochondrial Temperature Using Charged Thermosensitive Fluorophores (2020) (12)
Liver cytochrome c oxidase deficiency in a case of neonatal-onset hepatic failure (1994) (12)
Induced pluripotent stem cells-derived neurons from patients with Friedreich ataxia exhibit differential sensitivity to resveratrol and nicotinamide (2019) (11)
Iron overload and mitochondrial diseases (1998) (11)
Prenatal diagnosis of cytochrome c oxidase deficiency in cultured amniocytes is hazardous (1992) (11)
DAPIT Over-Expression Modulates Glucose Metabolism and Cell Behaviour in HEK293T Cells (2015) (11)
Paradoxical Inhibition of Glycolysis by Pioglitazone Opposes the Mitochondriopathy Caused by AIF Deficiency (2017) (11)
Sequence variations in the NDUFA1 gene encoding a subunit of complex I of the respiratory chain (2001) (11)
Phenotypic expression of mitochondrial genotypes in cultured skin fibroblasts and in Epstein–Barr virus–transformed lymphocytes in Pearson syndrome (1995) (11)
Processing of the 24 kDa subunit mitochondrial import signal is not required for assembly of functional complex I in Yarrowia lipolytica. (2004) (10)
The Effect of Adenine Nucleotides on Purified Phosphoenolpyruvate Carboxylase from the CAM Plant Crassula argentea. (1988) (10)
Maple syrup urine disease metabolites induce apoptosis in neural cells without cytochrome c release or changes in mitochondrial membrane potential. (1998) (10)
The control of malate dehydrogenase activity by adenine nucleotides in purified potato tuber (Solanum tuberosum L.) mitochondria. (1986) (10)
Mitochondria and cancer. (2007) (10)
CHCHD2 accumulates in distressed mitochondria and facilitates oligomerization of CHCHD10. (2018) (10)
Diffuse leukodystrophy in an infant with cytochrome-c oxidase deficiency (1998) (10)
Novel FH mutation in a patient with cutaneous leiomyomatosis associated with cutis verticis gyrata, eruptive collagenoma and Charcot‐Marie‐Tooth disease (2010) (10)
miR-379 links glucocorticoid treatment with mitochondrial response in Duchenne muscular dystrophy (2020) (10)
Developmental aspects of respiratory chain from fetus to infancy. (2011) (9)
Changing the diet to make more mitochondria and protect the heart. (2012) (9)
Report on three additional patients and genotype–phenotype correlation in SLC25A22-related disorders group (2019) (9)
Friedreich's Ataxia, Frataxin, PIP5K1B: Echo of a Distant Fracas (2013) (9)
Idebenone in Friedreich ataxia and Leber's hereditary optic neuropathy: close mechanisms, similar therapy? (2016) (8)
An Effective, Versatile, and Inexpensive Device for Oxygen Uptake Measurement (2017) (8)
[Metabolic and genetic investigations in childhood cardiomyopathies]. (1999) (8)
Mitochondrial Respiratory Chain (2008) (8)
Different pathophysiological mechanisms of intramitochondrial iron accumulation in acquired and congenital sideroblastic anemia caused by mitochondrial DNA deletion (2006) (8)
Severe complex I deficiency in a case of neonatal‐onset lactic acidosis and fatal liver failure (1997) (8)
The deleterious G15498A mutation in mitochondrial DNA-encoded cytochrome b may remain clinically silent in homoplasmic carriers (2004) (7)
The Human Cytochrome c Oxidase Assembly Factors SCO1 and SCO2 Have Regulatory Roles in the Maintenan (2007) (7)
[Targeting allotopic material to the mitochondrial compartment: new tools for better understanding mitochondrial physiology and prospect for therapy]. (2007) (7)
In Vitro Assessment of Mitochondrial Toxicity to Predict Drug-Induced Liver Injury (2018) (7)
Sequence and structure of the human OXA1L gene and its upstream elements. (1997) (7)
Mitochondrial cytopathies, phenotypic heterogeneity and a high incidence. (2006) (7)
Mitochondrial DNA inheritance in patients with deleted mtDNA (2001) (7)
Arabidopsis thaliana alternative dehydrogenases: a potential therapy for mitochondrial complex I deficiency? Perspectives and pitfalls (2019) (7)
Succinate dehydrogenase in KIT/PDGFRA wild-type gastrointestinal stromal tumors. (2010) (6)
Mitochondrial dysfunction in HIV infection: an overview of pathogenesis. (2001) (6)
Screening human EST database for identification of candidate genes in respiratory chain deficiency. (2000) (5)
Determination of enzyme activities for prenatal diagnosis of respiratory chain deficiency. (2000) (5)
Discrimination between duroquinol oxidase activity and the terminal oxidation step of the cyanide-resistant electron transport pathway of plant mitochondria. (1986) (5)
Correction: DAPIT Over-Expression Modulates Glucose Metabolism and Cell Behaviour in HEK293T Cells (2015) (5)
Nicotinamide adenine dinucleotides permeate through mitochondrial membranes in human Epstein-Barr virus-transformed lymphocytes (1997) (5)
[Enzymatic activities of the mitochondrial respiratory chain in child cardiomyopathies. 34 cases prospectively studied by endomyocardial biopsy]. (1992) (5)
Neonatal seizures and limb malformations associated with liver-specific complex IV respiratory chain deficiency. (2004) (5)
Continuous measurement of oxaloacetate in purified mitochondria from the leaves of Kalanchoë blossfeldiana (1985) (4)
The Divalent Cation Pump and its Role in the Regulation of Malic Enzyme Activity in Purified Mitochondria from Potato Tuber (1989) (4)
Sulfate as an allosteric activator and competitive inhibitor of Crassula argentea phosphoenolpyruvate carboxylase (1989) (4)
A new threat identified in the use of SDHIs pesticides targeting the mitochondrial succinate dehydrogenase enzyme (2018) (4)
The Nature of the Terminal Oxidation Step of the Alternative Electron Transport Pathway (1987) (4)
Purification and spectral properties of Kalanchoe blossfeldiana leaf mitochondria (1984) (4)
ANT2-defective fibroblasts exhibit normal mitochondrial bioenergetics (2015) (4)
Drug development for mitochondrial disease: recent progress, current challenges, and future prospects (2016) (4)
Frataxin and Mitochondrial Iron (2002) (4)
Rare presentation of familial paraganglioma without evidence of mutation in the SDH, RET and VHL genes: towards further genetic heterogeneity (2009) (4)
[Mitochondrial disfunction and perinatal exposure to antiretroviral nucleoside analogues]. (2000) (4)
Mutations in BCS1, a mitochondrial respiratory chain assembly gene, are responsible for complex III deficiency in patients with tubulopathy, encephalopathy and liver failure. (2001) (4)
Distinctive Krebs cycle remodeling in iPSC-derived neural and mesenchymal stem cells. (2019) (3)
Mitochondria are physiologically maintained at close to (2018) (3)
25 MITOCHONDRIAL RESPIRATORY CHAIN DISORDERS (MRCD) PRESENTING AS LIVER DISEASE: DIVERSITY OF CLINICAL, BIOCHEMICAL AND HISTOLOGICAL FEATURES (1996) (3)
Correction: DAPIT Over-Expression Modulates Glucose Metabolism and Cell Behaviour in HEK293T Cells (2015) (3)
The Electron Transport Chain and Carcinogenesis (2009) (3)
Fluorescence Study of Chemical Modification of Phosphoenolpyruvate Carboxylase from Crassula argentea. (1991) (3)
PROPERTIES OF PERCOLL-PURIFIED MITOCHONDRIA FROM TUBERS OF MANIHOT ESCULENTA, A CYANOGENIC PLANT (1991) (2)
Succinate Dehydrogenase, Succinate, and Superoxides: A Genetic, Epigenetic, Metabolic, Environmental Explosive Crossroad (2022) (2)
Cyanide resistant respiration and the alternative oxidase pathway: A journey from plants to mammals. (2022) (2)
Succinate-driven reverse electron transport in plant mitochondria. The effect of added NAD+ and the potential role of external malate dehydrogenase (1989) (2)
Expression study of genes involved in iron metabolism in human tissues. (2001) (2)
diverse phenotypes due to cytochrome oxidase deficiency (2013) (2)
Isolation, purification and photosynthetic activity of chloroplasts from Kalanchoe blossfeldiana, a CAM-performing plant (1986) (2)
The effect of chemical modification by diethylpyrocarbonate on substrate and effector binding to phosphoenolpyruvate carboxylase from Crassula argentea. Evidence for a histidine residue in the malate binding site (1993) (2)
[Friedreich's ataxia and mitochondria: the puzzle reconstructed]. (1999) (1)
[Friedreich ataxia. 3 years after the identification of the gene a glimmer of hope for therapy]. (2008) (1)
Redox Properties of Inhibitors of the Alternative Pathway of Plant Mitochondria (1987) (1)
Association of TCF7L2 variation with single islet autoantibody expression in children (2002) (1)
The Redox State of Mitochondrial Pyridine Nucleotides Versus Rate of Substrate Oxidation (1987) (1)
Production of Mitochondrial Reactive Oxygen Species Protein in Hypoxia Occurs Independently α Stabilization of Hypoxia-inducible Factor-1 Cell Biology : (2010) (0)
miR-379 links glucocorticoid treatment with mitochondrial response in Duchenne muscular dystrophy (2020) (0)
Relevance of the TRIAP1/p53 axis in colon cancer cell proliferation and adaptation to glutamine deprivation (2022) (0)
A Faithful Model of Optic Neuropathy Created by an Induced Nd4 Gene Heteroplasmy (2010) (0)
Effect of idebenone on cardiomy o p a t hy in Fr i e d r e i c h 's ataxia: a p r e l i m i n a ry study (1999) (0)
Long-term Transgene Expression And Safety Of a rAAV2 Approach Of Gene Therapy For Leber Hereditary Optic Neuropathy (2011) (0)
Muscle-Speciﬁc Loss of Apoptosis-Inducing Factor Leads to Mitochondrial Dysfunction, Skeletal Muscle Atrophy, and Dilated Cardiomyopathy (2005) (0)
019 Neurological disease due to mitochondrial dysfunction and in utero exposure to antiretroviral nucleoside analogues (1999) (0)
Prediction of drug-induced liver injury in human with a multiparametric assay on the HepaRG® cell line (2013) (0)
Induced pluripotent stem cells-derived neurons from patients with Friedreich ataxia exhibit differential sensitivity to resveratrol and nicotinamide (2019) (0)
Harlequin Mouse: An Appropriate Model for Studying Progressive Visual Function Loss Due to Retinal Ganglion Cell and Photoreceptor Degeneration (2010) (0)
Enzymatic Properties of Phosphoenolpyruvate Carboxylase of Purified Chloroplasts from CAM-Performing Kalanchoe blossfeldiana Poelln. Plants (1986) (0)
16.9. Mitochondriopathies (2008) (0)
Succinate Dehydrogenase (SDH) Mutations in Patients with "Wild-Type" (Non-KIT, Non-PDGFRA-Mutated) Gastrointestinal Sarcomas (2010) (0)
Author ' s response to reviews Title : Limitations in high-throughput drug screening on a cellular model for Friedreich ataxia (2009) (0)
Week in The Journal F Cellular / Molecular cAMP and a Retinal Clock (2004) (0)
In vitro Studies on the Interactions in Malate Metabolism between Mitochondria and Chloroplasts from CAM-Performing Kalanchoe blossfeldiana (1987) (0)
[Heart failure from mitochondrial origin: a matter of diet?]. (2016) (0)
Friedreich Ataxia as an Example of the Dual Genome Control of Mitochondrial Function (2002) (0)
O.3MiR-379 link glucocorticoid treatment to mitochondrial dysfunction in Duchenne muscular dystrophy (2019) (0)
581 DISCUSSION Use of functional human cancer cell line mitochondria to explore the mechanisms of ABT-737-induced mitochondrial membrane permeabilization (2010) (0)
Abstract 1021: Use of functional tumor cell line mitochondria to explore the mechanisms of BH3 peptides and ABT-737-induced mitochondrial membrane permeabilization (2010) (0)
Familial deficiency in complex II of respiratory chain presenting as leukodystrophy (1992) (0)
Atrophy , and Dilated Cardiomyopathy Mitochondrial Dysfunction , Skeletal Muscle Apoptosis-Inducing Factor Leads to Muscle-Specific Loss of (2005) (0)
Special Issue: The evolving role of mitochondria in metabolism (2012) (0)
67 FAMILIAL INFANTILE CIRRHOSIS DUE TO DEFICIENCY OF RESPIRATORY CHAIN ENZYMES CODED BY MITOCHONDRIAL DNA (1994) (0)
[Mitochondrial disorders]. (2009) (0)
Mrna Localization to the Mitochondrial Surface: A Tool for the Treatment of Retinal Degeneration Due to Mitochondrial Genome Mutations (2007) (0)
HIF1 (cid:1) and pancreatic (cid:2) -cell development (2012) (0)
Expressed sequence tag database screening for identification of human genes. (2002) (0)
Fluorescence StudyofChemical Modification of Phosphoenolpyruvate Carboxylase fromCrassula argenteal (1991) (0)
TheEffect ofAdenine Nucleotides onPurified Phosphoenolpyruvate Carboxylase fromtheCAM Plant (1988) (0)
[A very, very short of breath HIV-infected patient]. (1999) (0)
285 Peripheral neuropathy as the predominant manifestation of a mitochondrial DNA deletion (1999) (0)
Clinical Aspects of Mitochon rial Disor ers (1992) (0)
Cytochrome b-560-deficient mitochondria from Daucus carota roots (1987) (0)

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What Schools Are Affiliated With Pierre Rustin?

Pierre Rustin is affiliated with the following schools: