Early signs of architectural and biomechanical failure in isolated myofibers and immortalized myoblasts from desmin-mutant knock-in mice.

doi:10.1038/s41598-017-01485-x

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Article

Early signs of architectural and biomechanical failure in isolated myofibers and immortalized myoblasts from desmin-mutant knock-in mice.

Scientific reports - 3 May 2017

Diermeier Stefanie, Iberl Julian, Vetter Kristina, Haug Michael, Pollmann Charlotte, Reischl Barbara, Buttgereit Andreas, Schürmann Sebastian, Spörrer Marina, Goldmann Wolfgang H, Fabry Ben, Elhamine Fatiha, Stehle Robert, Pfitzer Gabriele, Winter Lilli, Clemen Christoph S, Herrmann Harald, Schröder Rolf, Friedrich Oliver

Abstract excerpt

In striated muscle, desmin intermediate filaments interlink the contractile myofibrillar apparatus with mitochondria, nuclei, and the sarcolemma. The desmin network's pivotal role in myocytes is evident since mutations in the human desmin gene cause severe myopathies and cardiomyopathies. Here, we investigated skeletal muscle pathology in myofibers and myofibrils isolated from young hetero- and homozygous R349P...

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Identifiers

PMID: 28469177
DOI: 10.1038/s41598-017-01485-x
PMCID: PMC5431221

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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