Malgorzata Rak

发表

Unsuspected task for an old team: succinate, fumarate and other Krebs cycle acids in metabolic remodeling.

P. Bénit, E. Letouzé, P. Rustin, 2014, Biochimica et biophysica acta.

Biochemical consequences in yeast of the human mitochondrial DNA 8993T>C mutation in the ATPase6 gene found in NARP/MILS patients.

R. Kucharczyk, J. di Rago, Malgorzata Rak, 2009, Biochimica et biophysica acta.

Alternative Oxidase Expression in the Mouse Enables Bypassing Cytochrome c Oxidase Blockade and Limits Mitochondrial ROS Overproduction

P. Bénit, H. Jacobs, P. Gressens, 2013, PLoS genetics.

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.

P. Bénit, P. Rustin, Malgorzata Rak, 2011, Biochemical and biophysical research communications.

Mitochondria are physiologically maintained at close to 50 ̊C

P. Bénit, H. Jacobs, P. Rustin, 2018 .

Mitochondria Are Physiologically Maintained At Close To 50 C

P. Bénit, H. Jacobs, P. Rustin, 2017 .

Cyanide resistant respiration and the alternative oxidase pathway: A journey from plants to mammals.

P. Bénit, H. Jacobs, P. Rustin, 2022, Biochimica et biophysica acta. Bioenergetics.

Characterization of Gtf1p, the Connector Subunit of Yeast Mitochondrial tRNA-dependent Amidotransferase*

Malgorzata Rak, M. H. Barros, A. Tzagoloff, 2011, The Journal of Biological Chemistry.

F1-dependent translation of mitochondrially encoded Atp6p and Atp8p subunits of yeast ATP synthase

Malgorzata Rak, A. Tzagoloff, 2009, Proceedings of the National Academy of Sciences.

Mitochondria are physiologically maintained at close to 50 °C

P. Bénit, H. Jacobs, P. Rustin, 2017, bioRxiv.

Succinate Dehydrogenase, Succinate, and Superoxides: A Genetic, Epigenetic, Metabolic, Environmental Explosive Crossroad

P. Bénit, P. Rustin, A. Gimenez-Roqueplo, 2022, Biomedicines.

Evolutionarily conserved susceptibility of the mitochondrial respiratory chain to SDHI pesticides and its consequence on the impact of SDHIs on human cultured cells

P. Bénit, P. Gressens, L. Huc, 2019, PloS one.

ANT2-defective fibroblasts exhibit normal mitochondrial bioenergetics

P. Rustin, J. Vockley, Malgorzata Rak, 2015, Molecular genetics and metabolism reports.

Regulation of mitochondrial translation of the ATP8/ATP6 mRNA by Smt1p

Malgorzata Rak, A. Tzagoloff, R. Azpiroz, 2016, Molecular biology of the cell.

Mitochondrial complex I deficiency of nuclear origin II. Non-structural genes.

P. Bénit, P. Rustin, Malgorzata Rak, 2012, Molecular genetics and metabolism.

Arabidopsis thaliana alternative dehydrogenases: a potential therapy for mitochondrial complex I deficiency? Perspectives and pitfalls

P. Bénit, V. Tiranti, H. Prokisch, 2019, Orphanet Journal of Rare Diseases.

A new threat identified in the use of SDHIs pesticides targeting the mitochondrial succinate dehydrogenase enzyme

P. Bénit, P. Gressens, L. Huc, 2018, bioRxiv.

Mitochondria are physiologically maintained at close to

Howard T. Jacobs, Young-Tae Chang, P. Bénit, 2018 .

The pathogenic m.8993 T > G mutation in mitochondrial ATP6 gene prevents proton release from the subunit c-ring rotor of ATP synthase.

R. Kucharczyk, Nahia Ezkurdia, J. di Rago, 2021, Human molecular genetics.

Deregulating mitochondrial metabolite and ion transport has beneficial effects in yeast and human cellular models for NARP syndrome.

B. Salin, D. Caetano-Anollés, J. di Rago, 2019, Human molecular genetics.

Yeast Cells Lacking the Mitochondrial Gene Encoding the ATP Synthase Subunit 6 Exhibit a Selective Loss of Complex IV and Unusual Mitochondrial Morphology*

P. Slonimski, S. Duvezin-Caubet, B. Salin, 2007, Journal of Biological Chemistry.

Increasing Mitochondrial Substrate-level Phosphorylation Can Rescue Respiratory Growth of an ATP Synthase-deficient Yeast*

Piotr P Slonimski, M. Rigoulet, P. Slonimski, 2005, Journal of Biological Chemistry.

Assembly of F0 in Saccharomyces cerevisiae.

Malgorzata Rak, A. Tzagoloff, J. Brière, 2009, Biochimica et biophysica acta.

Mutations in CYC1, encoding cytochrome c1 subunit of respiratory chain complex III, cause insulin-responsive hyperglycemia.

P. Rustin, N. Laing, J. Christodoulou, 2013, American journal of human genetics.

The Putative GTPase Encoded by MTG3 Functions in a Novel Pathway for Regulating Assembly of the Small Subunit of Yeast Mitochondrial Ribosomes*

Malgorzata Rak, G. Alushin, M. H. Barros, 2012, The Journal of Biological Chemistry.

Mitochondrial ATP synthase disorders: molecular mechanisms and the quest for curative therapeutic approaches.

R. Kucharczyk, E. Couplan, M. Blondel, 2009, Biochimica et biophysica acta.

Yeast models of human mitochondrial diseases: from molecular mechanisms to drug screening

S. Duvezin-Caubet, J. di Rago, Malgorzata Rak, 2006, Biotechnology journal.

Supernumerary subunits NDUFA3, NDUFA5 and NDUFA12 are required for the formation of the extramembrane arm of human mitochondrial complex I

P. Rustin, Malgorzata Rak, 2014, FEBS letters.

Distinctive Krebs cycle remodeling in iPSC-derived neural and mesenchymal stem cells.

P. Bénit, P. Rustin, Malgorzata Rak, 2019, Biochemical and biophysical research communications.

ZIKA virus elicits P53 activation and genotoxic stress in human neural progenitors similar to mutations involved in severe forms of genetic microcephaly and p53

M. Vignuzzi, P. Gressens, F. Di Cunto, 2016, Cell Death and Disease.

ZIKA virus elicits P53 activation and genotoxic stress in human neural progenitors similar to mutations involved in severe forms of genetic microcephaly and p53

M. Vignuzzi, P. Gressens, F. Di Cunto, 2016, Cell Death and Disease.

SLC25A32 Mutations and Riboflavin-Responsive Exercise Intolerance.

P. Rustin, N. Romero, Malgorzata Rak, 2016, The New England journal of medicine.

Cis-silencing of PIP5K1B evidenced in Friedreich's ataxia patient cells results in cytoskeleton anomalies.

P. Rustin, Malgorzata Rak, L. Aubry, 2013, Human molecular genetics.

Mitochondrial cytochrome c oxidase deficiency.

P. Bénit, P. Rustin, Malgorzata Rak, 2016, Clinical science.

AIF-regulated oxidative phosphorylation supports lung cancer development

Peng Zhang, T. Köcher, O. Kepp, 2019, Cell Research.

Turnover of ATP synthase subunits in F1‐depleted HeLa and yeast cells

Masasuke Yoshida, Malgorzata Rak, A. Tzagoloff, 2011, FEBS letters.

, of Yeast Mitochondrial Ribosomes Regulating Assembly of the Small Subunit Functions in a Novel Pathway for MTG 3 The Putative GTPase Encoded by Cell Biology

Malgorzata Rak, G. Alushin, M. H. Barros, 2012 .

SLC13A3 variants cause acute reversible leukoencephalopathy and α‐ketoglutarate accumulation

A. Schlessinger, R. Helaers, R. Wevers, 2019, Annals of neurology.

Pitfalls in Monitoring Mitochondrial Temperature Using Charged Thermosensitive Fluorophores

P. Bénit, P. Rustin, G. Lenaers, 2020, Chemosensors.

A “Petite Obligate” Mutant of Saccharomyces cerevisiae

S. Duvezin-Caubet, J. di Rago, Malgorzata Rak, 2006, Journal of Biological Chemistry.

Paradoxical Inhibition of Glycolysis by Pioglitazone Opposes the Mitochondriopathy Caused by AIF Deficiency

P. Bénit, M. Zeviani, G. Kroemer, 2017, EBioMedicine.

Modular assembly of yeast mitochondrial ATP synthase

Malgorzata Rak, A. Tzagoloff, Malgorzata Rak, 2011, The EMBO journal.

A Yeast Model of the Neurogenic Ataxia Retinitis Pigmentosa (NARP) T8993G Mutation in the Mitochondrial ATP Synthase-6 Gene*

S. Duvezin-Caubet, Nahia Ezkurdia, J. di Rago, 2007, Journal of Biological Chemistry.

Depletion of WFS1 compromises mitochondrial function in hiPSC-derived neuronal models of Wolfram syndrome

S. Sarkar, D. Ward, Malgorzata Rak, 2023, Stem cell reports.

AIF-regulated oxidative phosphorylation supports lung cancer development

Peng Zhang, T. Köcher, O. Kepp, 2019, Cell Research.

Mutations in CYC1, encoding cytochrome c1 subunit of respiratory chain complex III, cause insulin-responsive hyperglycemia.

P. Rustin, N. Laing, J. Christodoulou, 2013, American journal of human genetics.

Assembly of F0 in Saccharomyces cerevisiae.

Malgorzata Rak, A. Tzagoloff, Xiaomei Zeng, 2009, Biochimica et biophysica acta.

An Effective, Versatile, and Inexpensive Device for Oxygen Uptake Measurement

P. Rustin, Malgorzata Rak, D. Chrétien, 2017, Journal of clinical medicine.