Cardiolipin Biosynthesis and Mitochondrial Energy Production in Pediatric Idiopathic Dilated Cardiomyopathy
小儿特发性扩张型心肌病的心磷脂生物合成和线粒体能量产生
基本信息
- 批准号:9242055
- 负责人:
- 金额:$ 16.52万
- 依托单位:
- 依托单位国家:美国
- 项目类别:
- 财政年份:2015
- 资助国家:美国
- 起止时间:2015-04-05 至 2020-02-29
- 项目状态:已结题
- 来源:
- 关键词:Acyl Coenzyme AAddressAdolescentAdrenergic beta-AgonistsAdultAnabolismAnimal ModelAppearanceAreaBasic ScienceBiochemicalBiogenesisBiological AssayBiologyCardiacCardiolipinsCardiovascular PhysiologyCardiovascular systemCarnitine Palmitoyltransferase IChildChildhoodCitrate (si)-SynthaseClinicalClinical SciencesColoradoComplexDataDiagnosisDietDilated CardiomyopathyDiseaseDisease OutcomeDoctor of PhilosophyElectron MicroscopyElectron TransportElectron Transport Complex IIIEnergy MetabolismEnsureEnvironmentEnzymesEtiologyExtramural ActivitiesFatty AcidsFellowshipFree Radical FormationFunctional disorderFundingGeneticGoalsHealthcare SystemsHeartHeart TransplantationHeart failureHumanImpairmentIsoproterenolLifeLinoleic AcidsMediator of activation proteinMedicalMentorsMentorshipMitochondriaModelingMolecularMusMyocardialMyocardial tissueMyocardiumOmega-6 Fatty AcidsOrganellesOutcomeOxidative StressPathologyPathway interactionsPatternPediatric HospitalsPediatric cardiologyPediatricsPerformancePharmacologyPhiladelphiaPhospholipidsPhysiciansPrimary idiopathic dilated cardiomyopathyPrincipal InvestigatorProcessProductionProgram DevelopmentRegulationResearchResidenciesResourcesRoleSamplingScientistSideStressSubgroupSystemTherapeutic InterventionTissue BanksTissuesTrainingTraining ProgramsTranslatingTransplanted tissueTreatment FailureUniversitiesUp-RegulationVulnerable PopulationsWorkacylcarnitinebasecareercostexperimental studyhealth care disparityhuman tissueimproved outcomeinterestlong chain fatty acidmembermitochondrial dysfunctionmouse modelnoveloxidationpediatric departmentpediatric heart failurepediatric idiopathic dilated cardiomyopathypediatric patientspersonalized approachprogramspublic health relevanceresponsesuccesstherapeutic targettranscription factortranslational medicine
项目摘要
DESCRIPTION (provided by applicant): This proposal describes a 5 year training program for the development of an academic career in the field pediatric heart failure. The principal investigator has MD, Ph.D. training, completed clinical residency training in Pediatrics and Genetics at the Children's Hospital of Philadelphia, and recently completed fellowship training in Pediatric Cardiology at the University of Colorado. During this period of clinical training she has
developed an interest in the etiologies of heart failure (HF) in children, and initiated a research
program under the primary mentorship of Dr. Brian Stauffer. The proposed research program will be the first to provide a thorough characterization of mitochondrial function and energy metabolism in pediatric idiopathic dilated cardiomyopathy (IDC). The project addresses a critical healthcare disparity in an under-represented and vulnerable population: children who have received little benefit from advances in adult HF treatments. The goal of the work is to impact outcomes of heart failure in children through identification of unique pathophysiologic processes in myocardium of children with IDC, allowing identification of dietary and pharmacologic therapies tailored to pediatric disease. The mentorship team provides a diverse training backgrounds and research expertise in cardiovascular biology. Each member is extramurally funded, a leader in the field of pediatric heart failure and cardiovascular physiology, and committed to their role as research mentors. Preliminary work has demonstrated evidence of changes in mitochondrial content, phospholipid content, and energy capacity in pediatric IDC. This research will focus on further characterization of mitochondrial dysfunction through analysis of stress and biogenesis responses and direct assay of the electron transport chain and mediators of fatty-acid influx for ß-oxidation using human tissue. Features of pediatric HF are recapitulated by treatment of a juvenile mouse with isoproterenol (ISO). This model is proposed as a model to further delineate the mechanisms of altered mitochondrial energy utilization in pediatric HF. The Department of Pediatrics at the University of Colorado Denver provides an ideal setting for training physician-scientists by incorporating expertise and resources from Clinical and Basic Science Departments throughout the University of Colorado System. This environment, with access to a large human heart tissue bank and the principal investigator's mentorship team are ideally suited to ensure the success of a novel area of research upon which a career in translational medicine can be constructed.
项目成果
期刊论文数量(0)
专著数量(0)
科研奖励数量(0)
会议论文数量(0)
专利数量(0)
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Kathryn C. Chatfield其他文献
Assessment of abnormal transvalvular flow and wall shear stress direction for pediatric/young adults with bicuspid aortic valve: A cross-sectional four-dimensional flow study
小儿/年轻成人二叶式主动脉瓣异常跨瓣血流和壁切应力方向的评估:一项横断面四维血流研究
- DOI:
10.1016/j.jocmr.2024.101102 - 发表时间:
2024-12-01 - 期刊:
- 影响因子:6.100
- 作者:
Takashi Fujiwara;LaDonna J. Malone;Kathryn C. Chatfield;Alex Berthusen;Brian Fonseca;Lorna P. Browne;Alex J. Barker - 通讯作者:
Alex J. Barker
Cardiomyopathy in a c.1528GC emHadha/em mouse is associated with cardiac tissue lipotoxicity and altered cardiolipin species
在c.1528G>C **E**noyl - CoA Hydratase/Dehydrogenase(ECHS1,文中误写为emHadha/em,推测可能是想表示这个基因)小鼠中的心肌病与心脏组织脂毒性以及心磷脂种类改变有关
- DOI:
10.1016/j.jlr.2025.100792 - 发表时间:
2025-05-01 - 期刊:
- 影响因子:4.100
- 作者:
Chibuike Eke;Shannon Babcock;Garen Gaston;Gabriela Elizondo;Hak Chung;Ayah Asal;Kathryn C. Chatfield;Genevieve C. Sparagna;Andrea E. DeBarber;William Packwood;Jonathan R. Lindner;Melanie B. Gillingham - 通讯作者:
Melanie B. Gillingham
Kathryn C. Chatfield的其他文献
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{{ truncateString('Kathryn C. Chatfield', 18)}}的其他基金
Cardiolipin Biosynthesis and Mitochondrial Energy Production in Pediatric Idiopathic Dilated Cardiomyopathy
小儿特发性扩张型心肌病的心磷脂生物合成和线粒体能量产生
- 批准号:
8869753 - 财政年份:2015
- 资助金额:
$ 16.52万 - 项目类别:
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