Paule Bénit
French Institute of Health and Medical Research
83Publications
34H-index
5,192Citations
Publications 83
Newest
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. Baraibar12
Estimated H-index: 12
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 Apr 1, 2018in Revue Francophone Des Laboratoires
Paule Bénit34
Estimated H-index: 34
(French Institute of Health and Medical Research),
Sylvie Bortoli6
Estimated H-index: 6
(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 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 Frank28
Estimated H-index: 28
2 Citations Source Cite
Published on Jan 25, 2018in PLOS Biology 9.16
Dominique Chretien45
Estimated H-index: 45
(French Institute of Health and Medical Research),
Paule Bénit34
Estimated H-index: 34
(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...
33 Citations Source Cite
Published on Mar 27, 2018in bioRxiv
Paule Bénit34
Estimated H-index: 34
(French Institute of Health and Medical Research),
Sylvie Bortoli6
Estimated H-index: 6
(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 Mar 1, 2017in EBioMedicine
Paule Bénit34
Estimated H-index: 34
(Paris Diderot University),
Alice Pelhaître1
Estimated H-index: 1
(Paris Diderot University)
+ 7 AuthorsPierre Rustin79
Estimated H-index: 79
(Paris Diderot University)
Mice with the hypomorphic AIF-Harlequin mutation exhibit a highly heterogeneous mitochondriopathy that mostly affects respiratory chain complex I, causing a cerebral pathology that resembles that found in patients with AIF loss-of-function mutations. Here we describe that the antidiabetic drug pioglitazone (PIO) can improve the phenotype of a mouse Harlequin (Hq) subgroup, presumably due to an inhibition of glycolysis that causes an increase in blood glucose levels. This glycolysis-inhibitory PI...
3 Citations Source Cite
Published on Dec 1, 2017in Scientific Reports 4.12
Lorena Fernández-Mosquera2
Estimated H-index: 2
,
Cátia V. Diogo11
Estimated H-index: 11
+ 7 AuthorsNuno Raimundo11
Estimated H-index: 11
Acute and chronic mitochondrial respiratory chain deficiency differentially regulate lysosomal biogenesis
11 Citations Source Cite
Published on Jun 8, 2017in Journal of Clinical Medicine
Paule Bénit34
Estimated H-index: 34
,
Dominique Chretien45
Estimated H-index: 45
+ 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....
2 Citations Source Cite
Published on May 1, 2016in M S-medecine Sciences 0.69
Paule Bénit34
Estimated H-index: 34
(French Institute of Health and Medical Research),
Pierre Rustin79
Estimated H-index: 79
(French Institute of Health and Medical Research)
Source Cite
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