Publications by authors named "Guy Lenaers"

99Publications

Secondary coenzyme q deficiency in neurological disorders.

Free Radic Biol Med 2021 Jan 12. Epub 2021 Jan 12.

Unité Mixte de Recherche (UMR) MITOVASC, Centre National de la Recherche Scientifique (CNRS) 6015, Institut National de la Santé et de la Recherche Médicale (INSERM) U1083, University of Angers, 49933 Angers, France; Neuromuscular Reference Center, Department of Neurology, CHU Angers, 49933, Angers, France. Electronic address:

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January 2021

Dominant optic atrophy: Culprit mitochondria in the optic nerve.

Prog Retin Eye Res 2020 Dec 16:100935. Epub 2020 Dec 16.

Molecular Genetics Laboratory, Institute for Ophthalmic Research, Center for Ophthalmology, University of Tübingen, Tübingen, Germany. Electronic address:

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December 2020

A rod-cone dystrophy is systematically associated to the RTN4IP1 recessive optic atrophy.

Retina 2020 Dec 7. Epub 2020 Dec 7.

MitoLab team, UMR CNRS 6015 - INSERM U1083, Institut MitoVasc, Angers University and Hospital, Angers, France.

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December 2020

A homozygous MPZL2 deletion is associated with non syndromic hearing loss in a moroccan family.

Int J Pediatr Otorhinolaryngol 2021 Jan 2;140:110481. Epub 2020 Nov 2.

Human Molecular Genetics Laboratory, Institut Pasteur du Maroc, Casablanca, Morocco. Electronic address:

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January 2021

Cancer/Testis Antigens into mitochondria: a hub between spermatogenesis, tumorigenesis and mitochondrial physiology adaptation.

Mitochondrion 2021 Jan 19;56:73-81. Epub 2020 Nov 19.

MitoLab Team, Institut MitoVasc, CNRS UMR6015, INSERM U1083, Angers University, Angers, France. Electronic address:

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January 2021

Dysfunctional T Cell Mitochondria Lead to Premature Aging.

Trends Mol Med 2020 09 22;26(9):799-800. Epub 2020 Jul 22.

Cellular Interactions and Therapeutic Applications (ICAT), Structure Féderative de Recherche (SFR) 4208, Université d'Angers, Angers, France; Host-Pathogen Interaction Study Group (GEIHP), Équipe d'Accueil (EA) 3142, Université d'Angers, Université de Bretagne Occidentale, Angers, France.

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September 2020

Mutations in the m-AAA proteases AFG3L2 and SPG7 are causing isolated dominant optic atrophy.

Neurol Genet 2020 Jun 20;6(3):e428. Epub 2020 May 20.

MitoLab Team (M.C., A.C., C.B., D.G., V.D.-D., S.L., V.P., P.R., D.B., P.A.-B., G.L.), UMR CNRS 6015-INSERM U1083, Institut MitoVasc, Angers University and Hospital; Genetics and immuno-cell therapy Team (M.C.), Mohammed First University, Oujda, Morocco; Departments of Biochemistry and Genetics (C.B., D.G., V.D.-D., E.C., V.P., P.R., D.B., P.A.-B.), University Hospital Angers; Department of Ophthalmology (A.M.), Centre Hospitalier Universitaire Saint-Pierre, Brussels, Belgium; Neuroophthalmology Department (C.V.), Rothschild Ophthalmologic Foundation, Paris; Exploration of Visual Function and Neuro-Ophthalmology Department (V.S., S.D.-D., I.D.B.), Lille University Hospital, Rue Emilie Laine, Lille Cedex; CHU Bordeaux (C.G.), Service de Génétique Médicale, Centre de Référence « Neurogénétique » and Université de Bordeaux, INSERM U 1211, Laboratoire Maladies Rares, Génétique et Métabolisme (MRGM) Bordeaux; School of Optometry and Vision Sciences (M.V.), Cardiff University and Cardiff Eye Unit, University Hospital of Wales; NIHR Biomedical Research Centre at Moorfields Eye Hospital and UCL Institute of Ophthalmology (N.J., P.Y.-W.-M.), London; Department of Clinical Neurosciences (P.Y.-W.-M.), Cambridge Centre for Brain Repair and MRC Mitochondrial Biology Unit, University of Cambridge; Cambridge Eye Unit (P.Y.-W.-M.), Addenbrooke's Hospital, Cambridge University Hospitals, UK; IRCCS Istituto Delle Scienze Neurologiche di Bologna (F.T., L.C., C.L.M., V.C.), Bellaria Hospital; Unit of Neurology (C.L.M., V.C.), Department of Biomedical and NeuroMotor Sciences (DIBINEM), University of Bologna, Italy; Centre de Compétence Maladies Rares (X.Z.), Clinique Pluridisciplinaire Jules Verne, Nantes; and National Centre in Rare Diseases (I.M.), Genetics of Sensory Diseases, University Hospital, Montpellier, France.

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June 2020

Data-Mining Approach on Transcriptomics and Methylomics Placental Analysis Highlights Genes in Fetal Growth Restriction.

Front Genet 2019 9;10:1292. Epub 2020 Jan 9.

Unité Mixte de Recherche (UMR) MITOVASC, Équipe Mitolab, Centre National de la Recherche Scientifique (CNRS) 6015, Institut National de la Santé et de la Recherche Médicale (INSERM) U1083, Université d'Angers, Angers, France.

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January 2020

Oxidative stress contributes differentially to the pathophysiology of Charcot-Marie-Tooth disease type 2K.

Exp Neurol 2020 01 23;323:113069. Epub 2019 Oct 23.

MitoLab, UMR CNRS 6015-INSERM 1083, MitoVasc Institute, University of Angers, Angers, France; University Hospital of Angers, Department of Biochemistry and Genetics, F-49100 Angers, France.

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January 2020

Metabolomics reveals highly regional specificity of cerebral sexual dimorphism in mice.

Prog Neurobiol 2020 01 23;184:101698. Epub 2019 Sep 23.

Département de Biochimie et Génétique, Centre Hospitalier Universitaire d'Angers, France; Equipe Mitolab, Unité Mixte de Recherche MITOVASC, CNRS 6015, INSERM U1083, Université d'Angers, Angers, France. Electronic address:

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January 2020

Warburg-like effect is a hallmark of complex I assembly defects.

Biochim Biophys Acta Mol Basis Dis 2019 09 20;1865(9):2475-2489. Epub 2019 May 20.

UMR CNRS 6015-INSERM U1083, MitoVasc Institute, University of Angers, Angers, France; Department of Biochemistry and Genetics, University Hospital of Angers, F-49000, France. Electronic address:

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September 2019

Metabolomic Profiling of Aqueous Humor in Glaucoma Points to Taurine and Spermine Deficiency: Findings from the Eye-D Study.

J Proteome Res 2019 03 11;18(3):1307-1315. Epub 2019 Feb 11.

Unité Mixte de Recherche MITOVASC, équipe Mitolab, Centre National de la Recherche Scientifique 6015, Institut National de la Santé et de la Recherche Médicale U1083 , Université d'Angers , Angers 49035 , France.

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March 2019

A Metabolomics Profiling of Glaucoma Points to Mitochondrial Dysfunction, Senescence, and Polyamines Deficiency.

Invest Ophthalmol Vis Sci 2018 09;59(11):4355-4361

Equipe Mitolab, Institut Mitovasc, Centre National de la Recherche Scientifique 6015, Institut National de la Santé et de la Recherche Médicale (INSERM) U1083, Université d'Angers, Angers, France.

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September 2018

Study of mitochondrial function in placental insufficiency.

Placenta 2018 07 17;67:1-7. Epub 2018 May 17.

MitoVasc Institute, UMR CNRS 6015, INSERM U1083, Angers, France; Department of Neonatal Medicine, CHU Angers, Angers, France. Electronic address:

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July 2018

Glycosylation as new pharmacological strategies for diseases associated with excessive angiogenesis.

Pharmacol Ther 2018 11 29;191:92-122. Epub 2018 Jun 29.

INSERM UMR1063, Stress Oxydant et Pathologies Métaboliques, Université d'Angers, Angers, France. Electronic address:

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November 2018

Alteration of Extracellular Nucleotide Metabolism in Pseudoxanthoma Elasticum.

J Invest Dermatol 2018 08 6;138(8):1862-1870. Epub 2018 Mar 6.

MITOVASC-UMR CNRS 6015 INSERM U1083, Angers University, France; University Hospital of Angers, Angers, France.

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August 2018

AP4 deficiency: A novel form of neurodegeneration with brain iron accumulation?

Neurol Genet 2018 Feb 24;4(1):e217. Epub 2018 Jan 24.

Département de Neuropédiatrie (A.R., B.E., P.M., F.R.), CHU Gui de Chauliac, Montpellier; Institut des Neurosciences de Montpellier (A.R., N.H., G.M., C.P.H.), INSERM U1051, Université de Montpellier; Service de Neuroradiologie (C.-J.R., N.L.), CHU Gui de Chauliac, Montpellier; Equipe MitoLab (M.C., G.L.), UMR CNRS 6015-INSERM 1083, Institut MitoVasc, University of Angers, France; Department of Medical Genetics (C. Goizet), Hopital Pellegrin, Bordeaux University Hospital; MRGM Laboratory (C. Goizet), INSERM U1211, University of Bordeaux; Laboratoire de Génétique Moléculaire (C. Guissart), CHU de Montpellier; U1046 INSERM (P.M., F.R.), UMR9214 CNRS, Université de Montpellier; Department of Neurology (C.M.), University Hospital Gui de Chauliac, Montpellier; Centre de Référence des Malformations et Maladies Congénitales du Cervelet (L.B.), Service de Génétique, Hôpital Armand Trousseau, AP-HP, Paris, France; Wellcome Trust Centre for Mitochondrial Research (R.H.), Institute of Genetic Medicine, Newcastle University, United Kingdom; and Centre of Reference for Genetic Sensory Diseases (C.P.H.), Montpellier, France.

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February 2018

OPA1: How much do we know to approach therapy?

Pharmacol Res 2018 05 15;131:199-210. Epub 2018 Feb 15.

Department of Pharmacy and Biotechnology (FABIT), University of Bologna, Bologna, Italy. Electronic address:

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May 2018

The accumulation of assembly intermediates of the mitochondrial complex I matrix arm is reduced by limiting glucose uptake in a neuronal-like model of MELAS syndrome.

Biochim Biophys Acta Mol Basis Dis 2018 May 14;1864(5 Pt A):1596-1608. Epub 2018 Feb 14.

UMR CNRS 6015-INSERM U1083, MitoVasc Institute, Angers University, Angers, France; Biochemistry and Genetics Department, Angers Hospital, F-49000, France. Electronic address:

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May 2018

OPA1 gene therapy prevents retinal ganglion cell loss in a Dominant Optic Atrophy mouse model.

Sci Rep 2018 02 6;8(1):2468. Epub 2018 Feb 6.

UMR INSERM U1051/Université Montpellier - Institut des Neurosciences de Montpellier, 34091, Montpellier, France.

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February 2018

Current mechanistic insights into the CCCP-induced cell survival response.

Biochem Pharmacol 2018 02 22;148:100-110. Epub 2017 Dec 22.

MitoLab, Mitochondrial Medicine Research Centre, UMR CNRS 6015-INSERM 1083, Institut MitoVasc, University of Angers, 49933 Angers, France. Electronic address:

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February 2018

Novel gene mutation in an atypical late-onset mitochondrial form of multifocal dystonia.

Neurol Genet 2017 Dec 11;3(6):e205. Epub 2017 Dec 11.

UMR CNRS 6015-INSERM U1083 (C.B., V.D.-D., N.G., D.G., M.B., D.B., P.A.-B., G.L., P.R., V.P.), Mitovasc Institute, Angers University, France; Department of Biochemistry and Genetics (C.B., V.D.-D., N.G., D.G., M.B., D.B., P.A.-B., G.L., P.R., V.P.), University Hospital of Angers, France; Department of Neurology (T.R.), University Hospital of Nantes, France; and CHU Reims (A.-S.L.), Hôpital Maison Blanche, Pole de biologie, Service de génétique, France.

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December 2017

Homozygous mutations in PJVK and MYO15A genes associated with non-syndromic hearing loss in Moroccan families.

Int J Pediatr Otorhinolaryngol 2017 Oct 21;101:25-29. Epub 2017 Jul 21.

Human Molecular Genetics Laboratory, Institut Pasteur du Maroc, Casablanca, Morocco. Electronic address:

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October 2017

Novel compound heterozygous mutations in the GPR98 (USH2C) gene identified by whole exome sequencing in a Moroccan deaf family.

Mol Biol Rep 2017 Oct 26;44(5):429-434. Epub 2017 Sep 26.

Human Molecular Genetics Laboratory, Institut Pasteur du Maroc, 1, Place Louis Pasteur, 20360, Casablanca, Morocco.

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October 2017

Neurotoxicity of Insecticides.

Curr Med Chem 2017 ;24(27):2988-3001

Département de Biochimie et Génétique, Centre Hospitalier Universitaire, 4 rue Larrey, Angers, F-49933. France.

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October 2017

Autophagy controls the pathogenicity of OPA1 mutations in dominant optic atrophy.

J Cell Mol Med 2017 10 4;21(10):2284-2297. Epub 2017 Apr 4.

PREMMi/Mitochondrial Medicine Research Centre, Institut MITOVASC, CNRS UMR 6015, INSERM U1083, Université d'Angers, CHU d'Angers, Angers, France.

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October 2017

A Nontargeted UHPLC-HRMS Metabolomics Pipeline for Metabolite Identification: Application to Cardiac Remote Ischemic Preconditioning.

Anal Chem 2017 02 6;89(3):2138-2146. Epub 2017 Jan 6.

PREMMi, Pôle de Recherche et d'Enseignement en Médecine Mitochondriale, Institut MITOVASC, CNRS 6214, INSERM U1083, Université d'Angers , 4 Rue Larrey, 49933 Angers CEDEX 9, France.

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February 2017

The addition of ketone bodies alleviates mitochondrial dysfunction by restoring complex I assembly in a MELAS cellular model.

Biochim Biophys Acta Mol Basis Dis 2017 01 1;1863(1):284-291. Epub 2016 Nov 1.

UMR CNRS 6214-INSERM U1083, Mitovasc Institute, Angers University, Angers, France; Biochemistry and Genetics Department, Angers Hospital, F-49000, France. Electronic address:

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January 2017

Increased mitochondrial fusion in a autosomal recessive CMT2A family with mitochondrial GTPase mitofusin 2 mutations.

J Peripher Nerv Syst 2016 12;21(4):365-369

PREMMi/Mitochondrial Medicine Research Centre, Institut MITOVASC, CNRS UMR 6214, INSERM U1083, Université d'Angers, CHU d'Angers, Angers, France.

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December 2016

Martinique Crinkled Retinal Pigment Epitheliopathy: Clinical Stages and Pathophysiologic Insights.

Ophthalmology 2016 10 26;123(10):2196-204. Epub 2016 Jul 26.

Institute for Neurosciences of Montpellier U1051, University of Montpellier - University Hospital, Genetics of Sensory Diseases, Montpellier, France. Electronic address:

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October 2016

WFS1 in Optic Neuropathies: Mutation Findings in Nonsyndromic Optic Atrophy and Assessment of Clinical Severity.

Ophthalmology 2016 09 7;123(9):1989-98. Epub 2016 Jul 7.

Maladies Sensorielles Génétiques, CHRU, Montpellier, France; INSERM U1051, Institute for Neurosciences of Montpellier, Montpellier, France; Université Montpellier, Montpellier, France. Electronic address:

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September 2016

REMOTE ISCHEMIC CONDITIONING INFLUENCES MITOCHONDRIAL DYNAMICS.

Shock 2016 Feb;45(2):192-7

*L'UNAM Université, Université d'Angers, EA 3860 Cardioprotection Remodelage et Thrombose †L'UNAM Université, Université d'Angers, INSERM U1083, CNRS UMR 6214 ‡CHU Angers, Service de Cardiologie §CHU Angers, Département de Biochimie et Génétique, Angers, France.

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February 2016

A novel mutation of AFG3L2 might cause dominant optic atrophy in patients with mild intellectual disability.

Front Genet 2015 19;6:311. Epub 2015 Oct 19.

Institut des Neurosciences de Montpellier, U1051 de l'INSERM, Université de Montpellier Montpellier, France ; PREMMi, CNRS UMR 6214 - INSERM U1083, Département de Biochimie et Génétique, Université d'Angers, CHU d'Angers Angers, France.

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November 2015

Recessive TBC1D24 Mutations Are Frequent in Moroccan Non-Syndromic Hearing Loss Pedigrees.

PLoS One 2015 15;10(9):e0138072. Epub 2015 Sep 15.

Laboratoire de Génétique Moléculaire Humaine, Département de Recherche Scientifique, Institut Pasteur du Maroc, Casablanca, Morocco.

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May 2016

A novel mutation in the TMC1 gene causes non-syndromic hearing loss in a Moroccan family.

Gene 2015 Dec 28;574(1):28-33. Epub 2015 Jul 28.

Laboratoire de Génétique Moléculaire Humaine, Département de Recherche Scientifique, Institut Pasteur du Maroc, Casablanca, Morocco. Electronic address:

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December 2015

In vivo time-lapse imaging of mitochondria in healthy and diseased peripheral myelin sheath.

Mitochondrion 2015 Jul 29;23:32-41. Epub 2015 May 29.

INSERM U1051, Institut des Neurosciences de Montpellier, Université de Montpellier, Montpellier 34091, France. Electronic address:

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July 2015

Mutation in NDUFA13/GRIM19 leads to early onset hypotonia, dyskinesia and sensorial deficiencies, and mitochondrial complex I instability.

Hum Mol Genet 2015 Jul 21;24(14):3948-55. Epub 2015 Apr 21.

Institut des Neurosciences de Montpellier, Université de Montpellier I et II, BP 74103, 34 091 Montpellier Cedex 5, France, Service de Neuropédiatrie, CHU Gui de Chauliac, 34 295 Montpellier, France,

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July 2015

Impairment of visual function and retinal ER stress activation in Wfs1-deficient mice.

PLoS One 2014 13;9(5):e97222. Epub 2014 May 13.

INSERM U1051, Institut des Neurosciences de Montpellier, France and Université de Montpellier I et II, Montpellier, France.

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December 2014

Mitochondrial fusion is frequent in skeletal muscle and supports excitation-contraction coupling.

J Cell Biol 2014 Apr 21;205(2):179-95. Epub 2014 Apr 21.

MitoCare Center, Department of Pathology, Anatomy, and Cell Biology, Thomas Jefferson University, Philadelphia, PA 19107.

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April 2014

Genetic and molecular analysis of the CLDN14 gene in Moroccan family with non-syndromic hearing loss.

Indian J Hum Genet 2013 Jul;19(3):331-6

Département de Recherche Scientifique, Laboratoire de Génétique Moléculaire et Humaine, Institut Pasteur, 1, Place Louis Pasteur, C.P. 20360 Casablanca, Morocco.

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July 2013

Analysis of CLDN14 gene in deaf Moroccan patients with non-syndromic hearing loss.

Gene 2013 Jul 13;523(1):103-5. Epub 2013 Apr 13.

Laboratoire de Génétique Moléculaire et Humaine, Département de Recherche Scientifique, Institut Pasteur du Maroc, 20100 Casablanca, Morocco.

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July 2013

[Neurotoxicity of pesticides: its relationship with neurodegenerative diseases].

Med Sci (Paris) 2013 Mar 27;29(3):273-8. Epub 2013 Mar 27.

Laboratoire récepteurs et canaux ioniques membranaires, UPRES EA 2647-USC INRA 1330, Université d'Angers, UFR sciences, 2, boulevard Lavoisier, 49045 Angers, France.

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March 2013

Why mitochondria must fuse to maintain their genome integrity.

Antioxid Redox Signal 2013 Aug 28;19(4):379-88. Epub 2013 Mar 28.

INSERM U1051, Institut des Neurosciences de Montpellier, Université de Montpellier I et II, Montpellier, France.

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August 2013

Dominant optic atrophy.

Orphanet J Rare Dis 2012 Jul 9;7:46. Epub 2012 Jul 9.

Institut des Neurosciences de Montpellier, U1051 de l'INSERM, Université de Montpellier I et II, BP 74103, F-34091 Montpellier cedex 05, France.

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July 2012

[Cellular senescence and the myth of Janus].

Med Sci (Paris) 2012 Mar 6;28(3):288-96. Epub 2012 Apr 6.

Inserm U844, Université de Montpellier Sud, Institut des neurosciences de Montpellier, Montpellier, France.

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March 2012

Molecular analysis of the TMPRSS3 gene in Moroccan families with non-syndromic hearing loss.

Biochem Biophys Res Commun 2012 Mar 20;419(4):643-7. Epub 2012 Feb 20.

Laboratoire de Génétique Moléculaire et Humaine, Département de Recherche Scientifique, Institut Pasteur du Maroc, 20360 Casablanca, Morocco.

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March 2012