Article

Fasting reverses PLN R14del-mediated cardiomyopathy through lysosomal reactivation

2026-03-26

Abstract excerpt

Genetic cardiomyopathies consist of a heterogeneous group of myocardial disorders caused by variants that disrupt key regulators of cardiac structure and function. Variants in PLN, encoding phospholamban (PLN), the main inhibitor of the sarco/endoplasmic reticulum Ca²⁺-ATPase 2a (SERCA2a), have been linked to both dilated cardiomyopathy (DCM) and arrhythmogenic cardiomyopathy (ACM). Among these, the PLN Arg14del...

Topics

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

Identifiers and source

Literature Corpus work: 470e030e-3fb6-5a78-a144-9e7e2d50f75a
DOI: 10.64898/2026.03.24.713684

Open publication

Related research

Semantic proximity does not establish scientific evidence.

Click a neighbor to travelStep 1 · 12 closest

Fasting reverses PLN R14del-mediated cardiomyopathy through lysosomal reactivationDOI 10.64898/2026.03.24.713684Read

The phospholamban R14del generates pathogenic aggregates by impairing autophagosome-lysosome fusion.Semantic proximity Read

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

Protein Aggregation Is an Early Manifestation of Phospholamban p.(Arg14del)-Related Cardiomyopathy: Development of PLN-R14del-Related Cardiomyopathy.Semantic proximity Read

Early consequences of the phospholamban mutation PLN-R14del+/- in a transgenic mouse model.Semantic proximity Read

Mitigation of Stress-induced Structural Remodeling and Functional Deficiency in iPSC-CMs with PLN R9C Mutation by Promoting AutophagySemantic proximity Read

Autophagy–Lysosomal Dysfunction as a Converging Mechanism of Cardiomyopathy in Lysosomal Storage Disorders: From Pathobiology to Targeted TherapySemantic proximity Read

A mutation in the human phospholamban gene, deleting arginine 14, results in lethal, hereditary cardiomyopathy.Semantic proximity Read

Compartmentalisation proteomics revealed endolysosomal protein network changes in a goat model of atrial fibrillationSemantic proximity Read

Characterization of the PLN p.Arg14del Mutation in Human Induced Pluripotent Stem Cell-Derived Cardiomyocytes.Semantic proximity Read

Promoting Autophagy Mitigates Stress-Induced Remodeling in Patient iPSC-CMs with the Phospholamban R9C Mutation.Semantic proximity Read

Ranolazine rescues the heart failure phenotype of PLN-deficient human pluripotent stem cell-derived cardiomyocytes.Semantic proximity Read

Perinuclear damage from nuclear envelope deterioration elicits stress responses that contribute to <i>LMNA</i> cardiomyopathySemantic proximity Read