grant
From Metabolic Stress to Energy Enhancement: Unraveling Complex V Dimerization in Mitochondrial Disorders
Organization COLUMBIA UNIVERSITY HEALTH SCIENCESLocation NEW YORK, UNITED STATESPosted 1 Aug 2025Deadline 30 Jun 2030
NIHUS FederalResearch GrantFY2025ATP SynthesisATP Synthesis PathwayATP50 ATP synthase subunitAddressAffectAmpullary CrestAssayBioassayBiochemicalBiochemistryBioenergeticsBiologicalBiological AssayBiological ChemistryBiologyBody TissuesCell BodyCell LineCell modelCellLineCellsCellular modelChildhoodClinicalCollaborationsComprehensionCrista ampullarisDNA mutationDataDefectDevelopmentDimerizationDiseaseDisorderDisorder of neurometabolic regulationDisorder of neurometabolism regulationDysfunctionDyskinesia SyndromesEnergy ExpenditureEnergy MetabolismEnzyme GeneEnzymesEquilibriumFRETFailureFibroblastsFluorescence Resonance Energy TransferFoundationsFunctional disorderFutureFörster Resonance Energy TransferGenesGeneticGenetic ChangeGenetic defectGenetic mutationGoalsH+ elementHumanHydrogen IonsHyperthermiaImaging ProceduresImaging TechnicsImaging TechniquesImpairmentIndividualInner mitochondrial membraneInterventionInvestigationKnock-outKnockoutLeigh DiseaseLeigh SyndromeLinkLiteratureMentorsMetabolicMetabolic DiseasesMetabolic DisorderMetabolic stressMethodologyMitochondriaMitochondrial ATP SynthaseMitochondrial DiseasesMitochondrial DisordersMitochondrial F(1)F(0) ATPaseMitochondrial Proton-Translocating ATPasesModern ManMolecularMolecular ConfigurationMolecular ConformationMolecular StereochemistryMovement Disorder SyndromesMovement DisordersMutateMutationNerve DegenerationNeurologicNeurologicalNeuron DegenerationOSCP proteinOrganellesOxidative PhosphorylationOxidative Phosphorylation PathwayPathogenicityPathologyPathway interactionsPatientsPeptidesPhysiciansPhysiologyPhysiopathologyProcessProductionProtein DimerizationProteinsProteomicsProtonsRNA SeqRNA sequencingRNAseqRegulationResearchRespiratory ChainRoleScientistStrains Cell LinesStructural defectStructural malformationStructureSubacute Necrotizing EncephalomyelitisSubacute Necrotizing EncephalomyelopathySubacute Necrotizing EncephalopathySyndromeSystemTechniquesTherapeuticThesaurismosisTimeTissuesVariantVariationVisualizationWorkbalancebalance functionbiologicbiological adaptation to stresscareercareer faircareer networkingcomplex Vconformationconformationalconformational stateconformationallyconformationscrista ampullacristaecultured cell linedevelopmentaldimergenome mutationimaging in vivoin vivo imaginginnovateinnovationinnovativeinsightknockout genemetabolism disordermitochondrialmonomerneural degenerationneurodegenerationneurodegenerativeneurological degenerationneurometabolic diseaseneurometabolic disorderneurometabolism diseaseneurometabolism disorderneuronal degenerationnew approachesnew therapeutic approachnew therapeutic interventionnew therapeutic strategiesnew therapy approachesnew treatment approachnew treatment strategynovelnovel approachesnovel strategiesnovel strategynovel therapeutic approachnovel therapeutic interventionnovel therapeutic strategiesnovel therapy approacholigomycin sensitivity-conferring proteinoverexpressoverexpressionpathophysiologypathwaypatient subclasspatient subclusterpatient subgroupspatient subpopulationspatient subsetspatient subtypespediatricprofessional networkingreaction; crisisresponseskillssocial rolespeed networkingstemstress responsestress; reactionstressorstructural abnormalitiesstructural anomaliessuperresolution microscopytooltranscriptome sequencingtranscriptomic sequencingtranslational investigatortranslational researchertranslational scientist
Description preview
Project Summary/Abstract:
Leigh syndrome (LS) is a neurometabolic syndrome characterized by severe and progressive regression, often
triggered by a variety of metabolic stressors. Despite LS represents the most frequent pediatric mitochondrial
disease, therapeutic options are limited and non-targeted. LS is associated with a wide array of genetic…
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