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The toxic effect of R350P mutant desmin in striated muscle of man and mouse.
Clemen CS, Stöckigt F, Strucksberg KH, Chevessier F, Winter L, Schütz J, Bauer R, Thorweihe JM, Wenzel D, Schlötzer-Schrehardt U, Rasche V, Krsmanovic P, Katus HA, Rottbauer W, Just S, Müller OJ, Friedrich O, Meyer R, Herrmann H, Schrickel JW, Schröder R. Clemen CS, et al. Among authors: herrmann h. Acta Neuropathol. 2015 Feb;129(2):297-315. doi: 10.1007/s00401-014-1363-2. Epub 2014 Nov 14. Acta Neuropathol. 2015. PMID: 25394388 Free PMC article.
Desminopathies: pathology and mechanisms.
Clemen CS, Herrmann H, Strelkov SV, Schröder R. Clemen CS, et al. Among authors: herrmann h. Acta Neuropathol. 2013 Jan;125(1):47-75. doi: 10.1007/s00401-012-1057-6. Epub 2012 Nov 11. Acta Neuropathol. 2013. PMID: 23143191 Free PMC article. Review.
Mutant desmin substantially perturbs mitochondrial morphology, function and maintenance in skeletal muscle tissue.
Winter L, Wittig I, Peeva V, Eggers B, Heidler J, Chevessier F, Kley RA, Barkovits K, Strecker V, Berwanger C, Herrmann H, Marcus K, Kornblum C, Kunz WS, Schröder R, Clemen CS. Winter L, et al. Among authors: herrmann h. Acta Neuropathol. 2016 Sep;132(3):453-73. doi: 10.1007/s00401-016-1592-7. Epub 2016 Jul 8. Acta Neuropathol. 2016. PMID: 27393313 Free PMC article.
Early signs of architectural and biomechanical failure in isolated myofibers and immortalized myoblasts from desmin-mutant knock-in mice.
Diermeier S, Iberl J, Vetter K, Haug M, Pollmann C, Reischl B, Buttgereit A, Schürmann S, Spörrer M, Goldmann WH, Fabry B, Elhamine F, Stehle R, Pfitzer G, Winter L, Clemen CS, Herrmann H, Schröder R, Friedrich O. Diermeier S, et al. Among authors: herrmann h. Sci Rep. 2017 May 3;7(1):1391. doi: 10.1038/s41598-017-01485-x. Sci Rep. 2017. PMID: 28469177 Free PMC article.
The MyoRobot technology discloses a premature biomechanical decay of skeletal muscle fiber bundles derived from R349P desminopathy mice.
Haug M, Meyer C, Reischl B, Prölß G, Vetter K, Iberl J, Nübler S, Schürmann S, Rupitsch SJ, Heckel M, Pöschel T, Winter L, Herrmann H, Clemen CS, Schröder R, Friedrich O. Haug M, et al. Among authors: herrmann h. Sci Rep. 2019 Jul 24;9(1):10769. doi: 10.1038/s41598-019-46723-6. Sci Rep. 2019. PMID: 31341183 Free PMC article.
Dual Functional States of R406W-Desmin Assembly Complexes Cause Cardiomyopathy With Severe Intercalated Disc Derangement in Humans and in Knock-In Mice.
Herrmann H, Cabet E, Chevalier NR, Moosmann J, Schultheis D, Haas J, Schowalter M, Berwanger C, Weyerer V, Agaimy A, Meder B, Müller OJ, Katus HA, Schlötzer-Schrehardt U, Vicart P, Ferreiro A, Dittrich S, Clemen CS, Lilienbaum A, Schröder R. Herrmann H, et al. Circulation. 2020 Dec;142(22):2155-2171. doi: 10.1161/CIRCULATIONAHA.120.050218. Epub 2020 Oct 7. Circulation. 2020. PMID: 33023321 Free article.
The desmin mutation R349P increases contractility and fragility of stem cell-generated muscle micro-tissues.
Spörrer M, Kah D, Gerum RC, Reischl B, Huraskin D, Dessalles CA, Schneider W, Goldmann WH, Herrmann H, Thievessen I, Clemen CS, Friedrich O, Hashemolhosseini S, Schröder R, Fabry B. Spörrer M, et al. Among authors: herrmann h. Neuropathol Appl Neurobiol. 2022 Apr;48(3):e12784. doi: 10.1111/nan.12784. Epub 2021 Dec 10. Neuropathol Appl Neurobiol. 2022. PMID: 34850968
1,807 results