Article
Mitochondrial dysfunction in human hypertrophic cardiomyopathy is linked to cardiomyocyte architecture disruption and corrected by improving NADH-driven mitochondrial respiration.
European heart journal - 1 Apr 2023
Nollet Edgar E, Duursma Inez, Rozenbaum Anastasiya, Eggelbusch Moritz, Wüst Rob C I, Schoonvelde Stephan A C, Michels Michelle, Jansen Mark, van der Wel Nicole N, Bedi Kenneth C, Margulies Kenneth B, Nirschl Jeff, Kuster Diederik W D, van der Velden Jolanda
Abstract excerpt
AIMS: Genetic hypertrophic cardiomyopathy (HCM) is caused by mutations in sarcomere protein-encoding genes (i.e. genotype-positive HCM). In an increasing number of patients, HCM occurs in the absence of a mutation (i.e. genotype-negative HCM). Mitochondrial dysfunction is thought to be a key driver of pathological remodelling in HCM. Reports of mitochondrial respiratory function and specific disease-modifying...
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