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Pierre Rustin
French Institute of Health and Medical Research
337Publications
79H-index
23.3kCitations
Publications 337
Newest
Sarah Benlamara (French Institute of Health and Medical Research), Laetitia Aubry8
Estimated H-index: 8
(French Institute of Health and Medical Research)
+ 3 AuthorsMalgorzata Rak6
Estimated H-index: 6
(French Institute of Health and Medical Research)
Abstract Mitochondria play a vital role in proliferation and differentiation and their remodeling in the course of differentiation is related to the variable energy and metabolic needs of the cell. In this work, we show a distinctive mitochondrial remodeling in human induced pluripotent stem cells differentiated into neural or mesenchymal progenitors. While leading to upregulation of the citrate synthase-α-ketoglutarate dehydrogenase segment of the Krebs cycle and increased respiratory chain act...
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Published on Apr 1, 2019in Biochimica et Biophysica Acta 3.44
Sina Saari2
Estimated H-index: 2
(University of Tampere),
Geovana S. Garcia1
Estimated H-index: 1
(Sao Paulo State University)
+ 9 AuthorsPierre Rustin79
Estimated H-index: 79
(French Institute of Health and Medical Research)
Abstract The alternative respiratory chain (aRC), comprising the alternative NADH dehydrogenases (NDX) and quinone oxidases (AOX), is found in microbes, fungi and plants, where it buffers stresses arising from restrictions on electron flow in the oxidative phosphorylation system. The aRC enzymes are also found in species belonging to most metazoan phyla, including some chordates and arthropods species, although not in vertebrates or in Drosophila . We postulated that the aRC enzymes might be dep...
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Published on Jan 3, 2019in The EMBO Journal 10.56
Talha Rashid1
Estimated H-index: 1
,
Ivan Nemazanyy13
Estimated H-index: 13
+ 16 AuthorsMartin A. Baraibar13
Estimated H-index: 13
Abstract As a consequence of impaired glucose or fatty acid metabolism, bioenergetic stress in skeletal muscles may trigger myopathy and rhabdomyolysis. Genetic mutations causing loss of function of the LPIN1 gene frequently lead to severe rhabdomyolysis bouts in children, though the metabolic alterations and possible therapeutic interventions remain elusive. Here, we show that lipin1 deficiency in mouse skeletal muscles is sufficient to trigger myopathy. Strikingly, muscle fibers display strong...
1 Citations Source Cite
Published on Aug 28, 2018in Human Molecular Genetics 4.90
Xiaoping Huang1
Estimated H-index: 1
(National Institutes of Health),
Beverly Wu2
Estimated H-index: 2
(National Institutes of Health)
+ 8 AuthorsStephan Frank29
Estimated H-index: 29
3 Citations Source Cite
Published on Apr 1, 2018in Neurology Genetics
Cecilia Marelli19
Estimated H-index: 19
,
Christian P. Hamel41
Estimated H-index: 41
+ 8 AuthorsMichel Koenig49
Estimated H-index: 49
Aconitase 2 ( ACO2 ) encodes the mitochondrial aconitase (ACO2), an enzyme catalyzing interconversion of citrate into isocitrate in the Krebs cycle. ACO2 mutations have been initially associated with infantile cerebellar-retinal degeneration combining optic atrophy, retinal degeneration, severe encephalopathy, epilepsy, and cerebellar ataxia1–3; subsequently, ACO2 mutations have also been associated with milder presentations including isolated optic atrophy2 or cerebellar ataxia without optic at...
4 Citations Source Cite
Published on Apr 1, 2018in Revue Francophone Des Laboratoires
Paule Bénit35
Estimated H-index: 35
(French Institute of Health and Medical Research),
Sylvie Bortoli7
Estimated H-index: 7
(Paris Descartes University)
+ 2 AuthorsPierre Rustin79
Estimated H-index: 79
(French Institute of Health and Medical Research)
Resume Chez l'homme, les dysfonctions des enzymes du cycle de Krebs liees a des mutations causent des encephalopathies severes du jeune enfant, ou differents types de tumeurs et de cancers touchant differents organes. Ainsi des mutations des genes de la succinate-deshydrogenase ont ete identifiees dans des formes familiales de pheochromocytomes et paragangliomes. Les mutations du gene de la fumarase s'observent dans des leiomyomes uterins en association avec des cancers renaux, mais des cas d'en...
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Published on Mar 27, 2018in bioRxiv
Paule Bénit35
Estimated H-index: 35
(French Institute of Health and Medical Research),
Sylvie Bortoli7
Estimated H-index: 7
(Paris Descartes University)
+ 6 AuthorsPierre Rustin79
Estimated H-index: 79
(French Institute of Health and Medical Research)
Succinate dehydrogenase inhibitors (SDHIs) are now widely used worldwide as fungicides to limit the proliferation of molds in cereal crops, or to better preserve fruits, vegetables, and seeds from these molds, as well as to facilitate the lawn care for public spaces and golf courses. According to the companies that produce them, the SDHIs quite specifically inhibit the activity of the succinate dehydrogenase in the molds. We here establish that these inhibitors readily inhibit the earthworm and ...
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Published on Jan 25, 2018in PLOS Biology 9.16
Dominique Chrétien1
Estimated H-index: 1
(French Institute of Health and Medical Research),
Paule Bénit35
Estimated H-index: 35
(French Institute of Health and Medical Research)
+ 7 AuthorsMalgorzata Rak4
Estimated H-index: 4
(French Institute of Health and Medical Research)
In endothermic species, heat released as a product of metabolism ensures stable internal temperature throughout the organism, despite varying environmental conditions. Mitochondria are major actors in this thermogenic process. Part of the energy released by the oxidation of respiratory substrates drives ATP synthesis and metabolite transport, but a substantial proportion is released as heat. Using a temperature-sensitive fluorescent probe targeted to mitochondria, we measured mitochondrial tempe...
38 Citations Source Cite
Published on Jan 1, 2018
Mathieu Porceddu5
Estimated H-index: 5
,
Nelly Buron6
Estimated H-index: 6
+ 2 AuthorsAnnie Borgne-Sanchez9
Estimated H-index: 9
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Published on Jun 8, 2017in Journal of Clinical Medicine
Paule Bénit35
Estimated H-index: 35
,
Dominique Chretien46
Estimated H-index: 46
+ 3 AuthorsPierre Rustin79
Estimated H-index: 79
In the last ten years, the use of fluorescent probes developed to measure oxygen has resulted in several marketed devices, some unreasonably expensive and with little flexibility. We have explored the use of the effective, versatile, and inexpensive Redflash technology to determine oxygen uptake by a number of different biological samples using various layouts. This technology relies on the use of an optic fiber equipped at its tip with a membrane coated with a fluorescent dye (www.pyro-science....
3 Citations Source Cite
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