0000000000866886

AUTHOR

Philippe Mateo

showing 3 related works from this author

Impaired Binding to Junctophilin-2 and Nanostructural Alteration in CPVT Mutation

2021

Rationale: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare disease, manifested by syncope or sudden death in children or young adults under stress conditions. Mutations in the Ca 2+ release channel/RyR2 (type 2 ryanodine receptor) gene account for about 60% of the identified mutations. Recently, we found and described a mutation in RyR2 N-terminal domain, RyR2 R420Q . Objective: To determine the arrhythmogenic mechanisms of this mutation. Methods and Results: Ventricular tachycardias under stress conditions were observed in both patients with catecholaminergic polymorphic ventricular tachycardia and knock-in mice. During action potential recording (by patch-clamp in …

Ile de francePhysiologyCPVT030204 cardiovascular system & hematologyArticle03 medical and health sciences0302 clinical medicineaction potential[SDV.MHEP.CSC]Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular systemPolitical sciencejunctophilinryanodine receptormedia_common.cataloged_instanceHumansEuropean union610 Medicine & health030304 developmental biologymedia_common0303 health sciencescalciumRyanodine Receptor Calcium Release ChannelRyR2musculoskeletal systemSarcoplasmic ReticulumDeath Sudden Cardiaccalcium induced calcium releaseGain of Function Mutationcardiomyocyte calcium handlingcardiovascular systemventricular tachycardiamutationCardiology and Cardiovascular MedicineHumanities
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Down-regulation of OPA1 alters mouse mitochondrial morphology, PTP function, and cardiac adaptation to pressure overload

2012

AIMS: The optic atrophy 1 (OPA1) protein is an essential protein involved in the fusion of the mitochondrial inner membrane. Despite its high level of expression, the role of OPA1 in the heart is largely unknown. We investigated the role of this protein in Opa1(+/-) mice, having a 50% reduction in OPA1 protein expression in cardiac tissue. METHODS AND RESULTS: In mutant mice, cardiac function assessed by echocardiography was not significantly different from that of the Opa1(+/+). Electron and fluorescence microscopy revealed altered morphology of the Opa1(+/-) mice mitochondrial network; unexpectedly, mitochondria were larger with the presence of clusters of fused mitochondria and altered c…

Cardiac function curveendocrine systemPhysiologyAdaptation BiologicalDown-RegulationBiologyMitochondrionMitochondrial Membrane Transport ProteinsPermeabilityGTP PhosphohydrolasesMitochondrial ProteinsMice03 medical and health sciencesMitochondrial membrane transport protein0302 clinical medicinePhysiology (medical)Optic Atrophy Autosomal DominantPressuremedicineAnimalsMyocyteMyocytes CardiacInner mitochondrial membrane030304 developmental biologyMice KnockoutPressure overload0303 health sciencesMitochondrial Permeability Transition Poremedicine.diseaseeye diseasesMitochondriaCell biologyBiochemistryMitochondrial permeability transition poreMitochondrial Membranesbiology.proteinOptic Atrophy 1Cardiology and Cardiovascular Medicine030217 neurology & neurosurgeryCardiovascular Research
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Creatine kinase is the main target of reactive oxygen species in cardiac myofibrils.

1996

Abstract Reactive oxygen species (ROS) have been reported to alter cardiac myofibrillar function as well as myofibrillar enzymes such as myosin ATPase and creatine kinase (CK). To understand their precise mode and site of action in myofibrils, the effects of the xanthine/xanthine oxidase (X/XO) system or of hydrogen peroxide (H 2 O 2 ) have been studied in the presence and in the absence of phosphocreatine (PCr) in Triton X-100–treated cardiac fibers. We found that xanthine oxidase (XO), with or without xanthine, induced a decrease in maximal Ca 2+ -activated tension. We attributed this effect to the high contaminating proteolytic activity in commercial XO preparations, since it could be p…

Xanthine OxidasebiologyFree RadicalsPhysiologyMyosin ATPaseSuperoxideHydrogen PeroxideMyosinsXanthineMyocardial ContractionPhosphocreatineRatschemistry.chemical_compoundchemistryBiochemistryMyofibrilsbiology.proteinAnimalsCreatine kinasePMSFCardiology and Cardiovascular MedicineMyofibrilXanthine oxidaseReactive Oxygen SpeciesCreatine KinaseCirculation research
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