Engineered cardiac tissue model of restrictive cardiomyopathy for drug discovery

[1]  A. Karlin,et al.  Rad regulation of CaV1.2 channels controls cardiac fight-or-flight response , 2022, Nature Cardiovascular Research.

[2]  L. Mestroni,et al.  Activation of PDGFRA signaling contributes to filamin C–related arrhythmogenic cardiomyopathy , 2022, Science advances.

[3]  Swithin S. Razu,et al.  Subcellular Remodeling in Filamin C Deficient Mouse Hearts Impairs Myocyte Tension Development during Progression of Dilated Cardiomyopathy , 2022, International journal of molecular sciences.

[4]  Olaia F. Vila,et al.  milliPillar: A Platform for the Generation and Real-Time Assessment of Human Engineered Cardiac Tissues. , 2021, ACS biomaterials science & engineering.

[5]  Jourdan K. Ewoldt,et al.  Filamin C Cardiomyopathy Variants Cause Protein and Lysosome Accumulation , 2021, Circulation research.

[6]  C. Mummery,et al.  Cardiac Tissues From Stem Cells , 2021, Circulation research.

[7]  N. Frey,et al.  Cardiac Filaminopathies: Illuminating the Divergent Role of Filamin C Mutations in Human Cardiomyopathy , 2021, Journal of clinical medicine.

[8]  Nikolaos G. Frangogiannis,et al.  Cardiac fibrosis. , 2020, Cardiovascular research.

[9]  Catherine L. Worth,et al.  Cells of the adult human heart , 2020, Nature.

[10]  D. Judge,et al.  Epidemiology of the inherited cardiomyopathies , 2020, Nature Reviews Cardiology.

[11]  Wesley L. McKeithan,et al.  Metabolic Maturation Media Improve Physiological Function of Human iPSC-Derived Cardiomyocytes , 2020, Cell reports.

[12]  James B. Hu,et al.  Wnt Activation and Reduced Cell-Cell Contact Synergistically Induce Massive Expansion of Functional Human iPSC-Derived Cardiomyocytes. , 2020, Cell stem cell.

[13]  C. Mummery,et al.  Human-iPSC-Derived Cardiac Stromal Cells Enhance Maturation in 3D Cardiac Microtissues and Reveal Non-cardiomyocyte Contributions to Heart Disease , 2020, Cell stem cell.

[14]  F. Nakamura,et al.  Structure and Function of Filamin C in the Muscle Z-Disc , 2020, International journal of molecular sciences.

[15]  Changming Tan,et al.  Loss of Filamin C Is Catastrophic for Heart Function. , 2020, Circulation.

[16]  S. Heymans,et al.  A mutation update for the FLNC gene in myopathies and cardiomyopathies , 2020, Human mutation.

[17]  Matthew J. Daniels,et al.  Mavacamten rescues increased myofilament calcium sensitivity and dysregulation of Ca2+ flux caused by thin filament hypertrophic cardiomyopathy mutations , 2020, American journal of physiology. Heart and circulatory physiology.

[18]  Joe Z. Zhang,et al.  Modelling diastolic dysfunction in induced pluripotent stem cell-derived cardiomyocytes from hypertrophic cardiomyopathy patients. , 2019, European heart journal.

[19]  C. Perret,et al.  FLNC pathogenic variants in patients with cardiomyopathies: Prevalence and genotype‐phenotype correlations , 2019, Clinical genetics.

[20]  J. Palomino-Doza,et al.  Missense Mutations in the FLNC Gene Causing Familial Restrictive Cardiomyopathy. , 2019, Circulation. Genomic and precision medicine.

[21]  S. Ware,et al.  Novel pathogenic variants in filamin C identified in pediatric restrictive cardiomyopathy , 2018, Human mutation.

[22]  H. Watkins,et al.  Hypertrophic cardiomyopathy mutations increase myofilament Ca2+ buffering, alter intracellular Ca2+ handling, and stimulate Ca2+-dependent signaling , 2018, The Journal of Biological Chemistry.

[23]  Chris Denning,et al.  CRISPR/Cas9 editing in human pluripotent stem cell-cardiomyocytes highlights arrhythmias, hypocontractility, and energy depletion as potential therapeutic targets for hypertrophic cardiomyopathy , 2018, European heart journal.

[24]  Stephen P. Ma,et al.  Advanced maturation of human cardiac tissue grown from pluripotent stem cells , 2018, Nature.

[25]  Godfrey L. Smith,et al.  MUSCLEMOTION , 2017, Circulation Research.

[26]  C. Dauphin,et al.  Truncating mutations on myofibrillar myopathies causing genes as prevalent molecular explanations on patients with dilated cardiomyopathy , 2017, Clinical genetics.

[27]  Robert W. Mills,et al.  Novel Mutation in FLNC (Filamin C) Causes Familial Restrictive Cardiomyopathy , 2017, Circulation. Cardiovascular genetics.

[28]  M. Gertz,et al.  Restrictive Cardiomyopathy: Genetics, Pathogenesis, Clinical Manifestations, Diagnosis, and Therapy. , 2017, Circulation research.

[29]  V. Álvarez,et al.  Screening of the Filamin C Gene in a Large Cohort of Hypertrophic Cardiomyopathy Patients , 2017, Circulation. Cardiovascular genetics.

[30]  J. Spudich,et al.  Early-Onset Hypertrophic Cardiomyopathy Mutations Significantly Increase the Velocity, Force, and Actin-Activated ATPase Activity of Human β-Cardiac Myosin. , 2016, Cell reports.

[31]  P. Noseworthy,et al.  Idiopathic Restrictive Cardiomyopathy Is Primarily a Genetic Disease , 2016 .

[32]  R. Bryson-Richardson,et al.  FLNC myofibrillar myopathy results from impaired autophagy and protein insufficiency. , 2016, Human molecular genetics.

[33]  J. Schwartzentruber,et al.  Mutations in FLNC are Associated with Familial Restrictive Cardiomyopathy , 2016, Human Mutation.

[34]  Nathaniel Huebsch,et al.  Automated Video-Based Analysis of Contractility and Calcium Flux in Human-Induced Pluripotent Stem Cell-Derived Cardiomyocytes Cultured over Different Spatial Scales. , 2015, Tissue engineering. Part C, Methods.

[35]  X. Puente,et al.  Mutations in filamin C cause a new form of familial hypertrophic cardiomyopathy , 2014, Nature Communications.

[36]  Praveen Shukla,et al.  Chemically defined generation of human cardiomyocytes , 2014, Nature Methods.

[37]  Gang Wang,et al.  Modeling the mitochondrial cardiomyopathy of Barth syndrome with iPSC and heart-on-chip technologies , 2014 .

[38]  Y. Liu,et al.  iPSC Reprogramming from Human Peripheral Blood Using Sendai Virus Mediated Gene Transfer , 2014 .

[39]  N. McMahon,et al.  Comparison of electrophysiological data from human-induced pluripotent stem cell-derived cardiomyocytes to functional preclinical safety assays. , 2013, Toxicological sciences : an official journal of the Society of Toxicology.

[40]  A. Marks,et al.  Calcium cycling proteins and heart failure: mechanisms and therapeutics. , 2013, The Journal of clinical investigation.

[41]  Raghav Venkataraman,et al.  Myofilament Ca Sensitization Increases Cytosolic Ca Binding Affinity, Alters Intracellular Ca Homeostasis, and Causes Pause-Dependent Ca-Triggered Arrhythmia , 2012, Circulation research.

[42]  M. Vorgerd,et al.  Filamin C-related myopathies: pathology and mechanisms , 2012, Acta Neuropathologica.

[43]  Gordon Keller,et al.  SIRPA is a specific cell-surface marker for isolating cardiomyocytes derived from human pluripotent stem cells , 2011, Nature Biotechnology.

[44]  L. Masterson,et al.  cAMP-dependent protein kinase A selects the excited state of the membrane substrate phospholamban. , 2011, Journal of molecular biology.

[45]  J. Potter,et al.  Correcting diastolic dysfunction by Ca2+ desensitizing troponin in a transgenic mouse model of restrictive cardiomyopathy. , 2010, Journal of molecular and cellular cardiology.

[46]  Masato Saito,et al.  Non-invasive characterization of mouse embryonic stem cell derived cardiomyocytes based on the intensity variation in digital beating video. , 2010, The Analyst.

[47]  J. Potter,et al.  Functional effects of a restrictive-cardiomyopathy-linked cardiac troponin I mutation (R145W) in transgenic mice. , 2009, Journal of molecular biology.

[48]  D. DeMets,et al.  Effects of low-dose oral enoximone administration on mortality, morbidity, and exercise capacity in patients with advanced heart failure: the randomized, double-blind, placebo-controlled, parallel group ESSENTIAL trials , 2009, European heart journal.

[49]  E. Arbustini,et al.  Restrictive cardiomyopathy , 2009, Current opinion in cardiology.

[50]  M. Vorgerd,et al.  The pathomechanism of filaminopathy: altered biochemical properties explain the cellular phenotype of a protein aggregation myopathy. , 2007, Human molecular genetics.

[51]  Haitao Wen,et al.  Thin Filament Disinhibition by Restrictive Cardiomyopathy Mutant R193H Troponin I Induces Ca2+-Independent Mechanical Tone and Acute Myocyte Remodeling , 2007, Circulation research.

[52]  L. Kunkel,et al.  Loss of FilaminC (FLNc) Results in Severe Defects in Myogenesis and Myotube Structure , 2006, Molecular and Cellular Biology.

[53]  R. Califf,et al.  Short-term intravenous milrinone for acute exacerbation of chronic heart failure: a randomized controlled trial. , 2002, JAMA.

[54]  D. Bers.  Cardiac excitation–contraction coupling , 2002, Nature.

[55]  A. Sonnenberg,et al.  Structural and functional aspects of filamins. , 2001, Biochimica et biophysica acta.

[56]  R H Hruban,et al.  Underlying causes and long-term survival in patients with initially unexplained cardiomyopathy. , 2000, The New England journal of medicine.

[57]  D. DeMets,et al.  Effect of oral milrinone on mortality in severe chronic heart failure. The PROMISE Study Research Group. , 1991, The New England journal of medicine.

[58]  R. Schlant,et al.  A comparison of oral milrinone, digoxin, and their combination in the treatment of patients with chronic heart failure. , 1989, The New England journal of medicine.