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Mitochondrial dysfunction in human hypertrophic cardiomyopathy is linked to cardiomyocyte architecture disruption and corrected by improving NADH-driven mitochondrial respiration

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 Jr; Margulies, Kenneth B; Nirschl, Jeff; Kuster, Diederik W D; van der Velden, Jolanda

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 …

2023-04-01AlgemeenArtikel

Intramuscular short-chain acylcarnitines in elderly people are decreased in (pre-)frail females, but not in males.

Marjanne van der Hoek (Onderzoeker); Arie Nieuwenhuizen (Onderzoeker); Ondřej Kuda (Onderzoeker); Paul Bos (Onderzoeker); Veronika Paluchová (Onderzoeker); Lars Verschuren (Onderzoeker); Anita van den Hoek (Onderzoeker); Robert Kleemann (Onderzoeker); Nic Veeger (Onderzoeker); Feike van der Leij (Lector); Jaap Keijer (Onderzoeker)

We tested the hypothesis that in human ageing a decreased intramuscular acylcarnitine status is associated with (pre-)frailty, reduced physical performance and altered mitochondrial function. Results showed that intramuscular total carnitine levels and acetylcarnitine levels were lower in (pre-)frail …

2020-06-15GezondheidArtikel

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Mitochondrial dysfunction in human hypertrophic cardiomyopathy is linked to cardiomyocyte architecture disruption and corrected by improving NADH-driven mitochondrial respiration

Intramuscular short-chain acylcarnitines in elderly people are decreased in (pre-)frail females, but not in males.

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