Article

A contraction stress model of hypertrophic cardiomyopathy due to thick filament sarcomere mutations

2018-09-04

Abstract excerpt

Thick filament sarcomere mutations are the most common cause of hypertrophic cardiomyopathy (HCM), a disorder of heart muscle thickening associated with sudden cardiac death and heart failure, with unclear mechanisms. We engineered an isogenic panel of four human HCM induced pluripotent stem cell (iPSc) models using CRISPR/Cas9, and studied iPSc-derived cardiomyocytes (iCMs) in 3-dimensional cardiac microtissue (C...

Topics

Open a Topic to create a Post that cites this publication.

Identifiers and source

Literature Corpus work: ccba0711-1eeb-543e-a6b3-e4e4dde512f7
DOI: 10.1101/408294

Open publication

Related research

Semantic proximity does not establish scientific evidence.

Click a neighbor to travelStep 1 · 12 closest

A contraction stress model of hypertrophic cardiomyopathy due to thick filament sarcomere mutationsDOI 10.1101/408294Read

A Contraction Stress Model of Hypertrophic Cardiomyopathy due to Sarcomere MutationsSemantic proximity Read

cMyBP-C ablation in human engineered cardiac tissue causes progressive Ca2+-handling abnormalities.Semantic proximity Read

Human iPSC-CMs and in-silico technologies define mechanisms and accelerate targeted pharmacogenetics in hypertrophic cardiomyopathySemantic proximity Read

Cardiomyopathy phenotypes caused by a heterozygous MYBPC3 mutation revealed in different hiPSC cardiac models.Semantic proximity Read

Mechanical Resistance to Micro-Heart Tissue Contractility unveils early Structural and Functional Pathology in iPSC Models of Hypertrophic CardiomyopathySemantic proximity Read

Hypertrophic cardiomyopathy-linked mutation in troponin T causes myofibrillar disarray and pro-arrhythmic action potential changes in human iPSC cardiomyocytes.Semantic proximity Read

Hypertrophic cardiomyopathy β-cardiac myosin mutation (P710R) leads to hypercontractility by disrupting super relaxed state.Semantic proximity Read

Long-term mavacamten exposure reduces force and sarcomere density in a hiPSC model of hypertrophic cardiomyopathySemantic proximity Read

Length-dependent hypercontractility decoupled from cellular adaptations is the initial phenotype in MYBPC3-haploinsufficient iPSC-derived engineered cardiac tissuesSemantic proximity Read

Mechanisms of Pathogenicity of Hypertrophic Cardiomyopathy-Associated Troponin T (TNNT2) Variant R278C <sup>+/-</sup> During DevelopmentSemantic proximity Read

Translational investigation of electrophysiology in hypertrophic cardiomyopathySemantic proximity Read

Development of a Cardiac Sarcomere Functional Genomics Platform to Enable Scalable Interrogation of Human TNNT2 Variants.Semantic proximity Read