Article

iPSC modeling shows uncompensated mitochondrial mediated oxidative stress underlies early heart failure in hypoplastic left heart syndrome

2021-05-10

Abstract excerpt

<h4>Summary</h4> Hypoplastic left heart syndrome (HLHS) is a severe congenital heart defect with 30% mortality from heart failure (HF) in the first year of life, but why only some patients suffer early-HF and its cause remain unknown. Modeling using induced pluripotent stem cell-derived cardiomyocytes (iPSC-CM) showed early-HF patient iPSC-CM have increased apoptosis, redox stress, and failed antioxidant response...

Topics

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

Identifiers and source

Literature Corpus work: 64807f78-c1b5-57f5-8977-e2b691b403e9
DOI: 10.1101/2021.05.09.443165

Open publication

Related research

Semantic proximity does not establish scientific evidence.

Click a neighbor to travelStep 1 · 12 closest

iPSC modeling shows uncompensated mitochondrial mediated oxidative stress underlies early heart failure in hypoplastic left heart syndromeDOI 10.1101/2021.05.09.443165Read

Sequential defects in cardiac lineage commitment and maturation cause hypoplastic left heart syndromeSemantic proximity Read

<i>LMNA</i> Haploinsufficiency in Human iPSC-Derived Cardiac Organoids Reveals Early Fibrotic Signaling as a Therapeutically Targetable ProcessSemantic proximity Read

Integrative single-cell and genetic profiling of human heart failure identifies targets for cardiomyocyte restorationSemantic proximity Read

Sequential Defects in Cardiac Lineage Commitment and Maturation Cause Hypoplastic Left Heart SyndromeSemantic proximity Read

Hypoplastic left heart syndrome cardiomyocytes exhibit intrinsic stress vulnerabilities and augmented stress responses in vitroSemantic proximity Read

Hypomorphic NOTCH1 Expression Alters Cardiomyocyte Cellular Architecture in Hypoplastic Left Heart SyndromeSemantic proximity Read

The single-cell atlas of the heart reveals an unexpected erythrocyte-like cardiomyocyteSemantic proximity Read

Updated Applications of Stem Cells in Hypoplastic Left Heart SyndromeSemantic proximity Read

Learning from failure: congestive heart failure in the postgenomic ageSemantic proximity Read

Human-induced pluripotent stem cells for modelling metabolic perturbations and impaired bioenergetics underlying cardiomyopathies.Semantic proximity Read

Impairment of the ER/mitochondria compartment in human cardiomyocytes with PLN p.Arg14del mutation.Semantic proximity Read

Unfolded Protein Response as a Compensatory Mechanism and Potential Therapeutic Target in PLN R14del Cardiomyopathy.Semantic proximity Read