2022
Muscle LIM Protein Force-Sensing Mediates Sarcomeric Biomechanical Signaling in Human Familial Hypertrophic Cardiomyopathy
Riaz M, Park J, Sewanan LR, Ren Y, Schwan J, Das SK, Pomianowski PT, Huang Y, Ellis MW, Luo J, Liu J, Song L, Chen IP, Qiu C, Yazawa M, Tellides G, Hwa J, Young LH, Yang L, Marboe CC, Jacoby DL, Campbell SG, Qyang Y. Muscle LIM Protein Force-Sensing Mediates Sarcomeric Biomechanical Signaling in Human Familial Hypertrophic Cardiomyopathy. Circulation 2022, 145: 1238-1253. PMID: 35384713, PMCID: PMC9109819, DOI: 10.1161/circulationaha.121.056265.Peer-Reviewed Original ResearchConceptsHypertrophic cardiomyopathySarcomeric mutationsFamilial hypertrophic cardiomyopathySudden cardiac deathCardiac myosin heavy chainMechanism-based treatmentsDevelopment of hypertrophyActivated T cellsCalcineurin-nuclear factorForce productionPhenotypic expressionPluripotent stem cell-derived cardiomyocytesStem cell-derived cardiomyocytesHeart failureCardiac deathVentricular hypertrophyCell-derived cardiomyocytesCardiac contractilityPharmacological interventionsT cellsCardiac diseaseCardiac hypertrophyPatient-specific induced pluripotent stem cellsPharmacological meansTwitch relaxation
2020
Subaortic Membranes in Patients With Hereditary Hemorrhagic Telangiectasia and Liver Vascular Malformations
Kim AS, Henderson KJ, Pawar S, Kim MJ, Punjani S, Pollak JS, Fahey JT, Garcia‐Tsao G, Sugeng L, Young LH. Subaortic Membranes in Patients With Hereditary Hemorrhagic Telangiectasia and Liver Vascular Malformations. Journal Of The American Heart Association 2020, 9: e016197. PMID: 33054561, PMCID: PMC7763373, DOI: 10.1161/jaha.120.016197.Peer-Reviewed Original ResearchMeSH KeywordsActivin Receptors, Type IICardiac Output, HighDiscrete Subaortic StenosisEchocardiographyFemaleHeart Defects, CongenitalHeart FailureHumansLiverMaleMiddle AgedMutationPrognosisRetrospective StudiesSurvival AnalysisTelangiectasia, Hereditary HemorrhagicUnited StatesVascular MalformationsConceptsHigh-output cardiac failureHereditary hemorrhagic telangiectasiaLeft ventricular outflow tractVentricular outflow tractHemorrhagic telangiectasiaMild obstructionSubaortic membraneVascular malformationsOutflow tractActivin receptor-like kinase 1 mutationsHereditary hemorrhagic telangiectasia patientsLiver vascular malformationsMild aortic insufficiencyPulmonary artery pressureRight heart catheterizationCohort of patientsRetrospective observational analysisHigh cardiac outputKinase 1 mutationsArtery pressureHeart catheterizationPulmonary hypertensionAortic insufficiencyBackground PatientsTricuspid regurgitation
2009
International guidelines for the diagnosis and management of hereditary haemorrhagic telangiectasia
Faughnan ME, Palda VA, Garcia-Tsao G, Geisthoff UW, McDonald J, Proctor DD, Spears J, Brown DH, Buscarini E, Chesnutt MS, Cottin V, Ganguly A, Gossage JR, Guttmacher AE, Hyland RH, Kennedy SJ, Korzenik J, Mager JJ, Ozanne AP, Piccirillo JF, Picus D, Plauchu H, Porteous ME, Pyeritz RE, Ross DA, Sabba C, Swanson K, Terry P, Wallace MC, Westermann CJ, White RI, Young LH, Zarrabeitia R. International guidelines for the diagnosis and management of hereditary haemorrhagic telangiectasia. Journal Of Medical Genetics 2009, 48: 73. PMID: 19553198, DOI: 10.1136/jmg.2009.069013.Peer-Reviewed Reviews, Practice Guidelines, Standards, and Consensus StatementsConceptsStructured consensus processGuideline processDiagnosis of HHTGuidelines Working GroupLife-threatening hemorrhageHealth care workersHereditary haemorrhagic telangiectasiaOvid MEDLINE databasesConsensus processSignificant arteriovenous malformationsAutosomal dominant diseaseSymptomatic diseaseSystematic search strategyWorking GroupConsensus guidelinesArteriovenous malformationsEvidence tablesClinic staffGuideline conferenceHealth care administratorsGastrointestinal tractAvailable screeningCare workersGuideline methodologistsMEDLINE databaseAMP‐activated protein kinase: a core signalling pathway in the heart
Kim AS, Miller EJ, Young LH. AMP‐activated protein kinase: a core signalling pathway in the heart. Acta Physiologica 2009, 196: 37-53. PMID: 19239414, DOI: 10.1111/j.1748-1716.2009.01978.x.BooksConceptsProtein kinaseEssential cellular processesTumor suppressor LKB1Downstream AMPK targetsProduction of ATPProtein phosphataseAMPK targetsActivated AMPKIntracellular glycogen accumulationCellular processesUpstream kinaseFatty acid metabolismCardiac myocyte hypertrophyAMPK activationAMPK activityImportant intracellularMolecular mechanismsMajor regulatorAMPKProtein synthesisKinaseAcid metabolismOral hypoglycaemic drugsGlycogen accumulationType 2 diabetes
2008
AMP-Activated Protein Kinase Conducts the Ischemic Stress Response Orchestra
Young LH. AMP-Activated Protein Kinase Conducts the Ischemic Stress Response Orchestra. Circulation 2008, 117: 832-840. PMID: 18268160, DOI: 10.1161/circulationaha.107.713115.Books