Results 1 to 10 of about 57,573 (289)

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

Scientific Reports, 2017
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 ...
Stefanie Diermeier, Julian Iberl, Kristina Vetter, Michael Haug, Charlotte Pollmann, Barbara Reischl, Andreas Buttgereit, Sebastian Schürmann, Marina Spörrer, Wolfgang H. Goldmann, Ben Fabry, Fatiha Elhamine, Robert Stehle, Gabriele Pfitzer, Lilli Winter, Christoph S. Clemen, Harald Herrmann, Rolf Schröder, Oliver Friedrich   +18 more
doaj   +2 more sources

Is Desmin Propensity to Aggregate Part of its Protective Function? [PDF]

Cells, 2020
Desmin is the major protein component of the intermediate filaments (IFs) cytoskeleton in muscle cells, including cardiac. The accumulation of cleaved and misfolded desmin is a cellular hallmark of heart failure (HF).
Sonia R. Singh, Hikmet Kadioglu, Krishna Patel, Lucie Carrier, Giulio Agnetti   +4 more
doaj   +2 more sources

Desmin forms toxic, seeding-competent amyloid aggregates that persist in muscle fibers [PDF]

Proceedings of the National Academy of Sciences of the United States of America, 2019
Desmin-associated myofibrillar myopathy (MFM) has pathologic similarities to neurodegeneration-associated protein aggregate diseases. Desmin is an abundant muscle-specific intermediate filament, and disease mutations lead to its aggregation in cells ...
Niraja Kedia, Mark Batchelor, Conrad C Weihl   +2 more
exaly   +3 more sources

Desmin-p.L112Q Disturbs Filament Formation and Is a Likely-Pathogenic Variant Associated with Dilated Cardiomyopathy [PDF]

Journal of Cardiovascular Development and Disease
DES encodes the muscle-specific intermediate filament protein desmin, which is highly relevant to the structural integrity of cardiomyocytes. Mutations in this gene cause different cardiomyopathies including dilated cardiomyopathy.
Alexander Lütkemeyer, Sabrina Voß, Jonas Reckmann, Joline Groß, Anna Gärtner, Jan Gummert, Hendrik Milting, Andreas Brodehl   +7 more
doaj   +2 more sources

Ubiquitylation by Trim32 causes coupled loss of desmin, Z-bands, and thin filaments in muscle atrophy [PDF]

, 2013
During muscle atrophy, myofibrillar proteins are degraded in an ordered process in which MuRF1 catalyzes ubiquitylation of thick filament components (Cohen et al. 2009. J. Cell Biol. http://dx.doi.org/10.1083/jcb.200901052).
Cohen, Shenhav Orit, Goldberg, Alfred L., Gygi, Steven P., Zhai, Bo   +3 more
core   +3 more sources

The myofibrillar myopathy-linked variant DES-p.T341P impairs desmin filament assembly. [PDF]

Mol Biol Rep
Lütkemeyer A, Voß S, Klag F, Groß J, Reckmann J, Gärtner A, Anselmetti D, Gummert J, Walhorn V, Milting H, Brodehl A.   +10 more
europepmc   +3 more sources

Desmin variants: Trigger for cardiac arrhythmias?

Frontiers in Cell and Developmental Biology, 2022
Desmin (DES) is a classical type III intermediate filament protein encoded by the DES gene. Desmin is abundantly expressed in cardiac, skeletal, and smooth muscle cells.
Wei Su, Wei Su, Stan W. van Wijk, Stan W. van Wijk, Bianca J. J. M. Brundel, Bianca J. J. M. Brundel   +5 more
doaj   +1 more source

Desmin Reorganization by Stimuli Inducing Oxidative Stress and Electrophiles: Role of Its Single Cysteine Residue

Antioxidants, 2023
The type III intermediate filament proteins vimentin and GFAP are modulated by oxidants and electrophiles, mainly through perturbation of their single cysteine residues.
Diego Moneo-Corcuera, Álvaro Viedma-Poyatos, Konstantinos Stamatakis, Dolores Pérez-Sala   +3 more
doaj   +1 more source

Global O-GlcNAcylation changes impact desmin phosphorylation and its partition toward cytoskeleton in C2C12 skeletal muscle cells differentiated into myotubes

Scientific Reports, 2022
Desmin is the guardian of striated muscle integrity, permitting the maintenance of muscle shape and the efficiency of contractile activity. It is also a key mediator of cell homeostasis and survival.
Charlotte Claeyssen, Bruno Bastide, Caroline Cieniewski-Bernard   +2 more
doaj   +1 more source

The In Vitro Cytotoxic Effects of Ionophore Exposure on Selected Cytoskeletal Proteins of C2C12 Myoblasts

Toxins, 2022
Carboxylic ionophores, such as monensin, salinomycin and lasalocid, are polyether antibiotics used widely in production animals for the control of coccidiosis, as well as for the promotion of growth and feed efficiency.
Danielle Henn, Annette Venter, Gezina C. H. Ferreira, Christo J. Botha   +3 more
doaj   +1 more source