Haug, Michael, Meyer, Charlotte, Reischl, Barbara, Proelss, Gerhard, Vetter, Kristina, Iberl, Julian, Nuebler, Stefanie, Schuermann, Sebastian, Rupitsch, Stefan J., Heckel, Michael, Poeschel, Thorsten, Winter, Lilli ORCID: 0000-0002-6368-1160, Herrmann, Harald, Clemen, Christoph S., Schroeder, Rolf and Friedrich, Oliver ORCID: 0000-0003-2238-2049 (2019). The MyoRobot technology discloses a premature biomechanical decay of skeletal muscle fiber bundles derived from R349P desminopathy mice. Sci Rep, 9. LONDON: NATURE PUBLISHING GROUP. ISSN 2045-2322

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Abstract

Mutations in the Des gene coding for the muscle-specific intermediate filament protein desmin lead to myopathies and cardiomyopathies. We previously generated a R349P desmin knock-in mouse strain as a patient-mimicking model for the corresponding most frequent human desmin mutation R350P. Since nothing is known about the age-dependent changes in the biomechanics of affected muscles, we investigated the passive and active biomechanics of small fiber bundles from young (17-23 wks), adult (25-45 wks) and aged (>60 wks) heterozygous and homozygous R349P desmin knock-in mice in comparison to wild-type littermates. We used a novel automated biomechatronics platform, the MyoRobot, to perform coherent quantitative recordings of passive (resting length-tension curves, visco-elasticity) and active (caffeine-induced force transients, pCa-force, 'slack-tests') parameters to determine age-dependent effects of the R349P desmin mutation in slow-twitch soleus and fast-twitch extensor digitorum longus small fiber bundles. We demonstrate that active force properties are not affected by this mutation while passive steady-state elasticity is vastly altered in R349P desmin fiber bundles compatible with a pre-aged phenotype exhibiting stiffer muscle preparations. Visco-elasticity on the other hand, was not altered. Our study represents the first systematic age-related characterization of small muscle fiber bundle preparation biomechanics in conjunction with inherited desminopathy.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Haug, MichaelUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Meyer, CharlotteUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Reischl, BarbaraUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Proelss, GerhardUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Vetter, KristinaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Iberl, JulianUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Nuebler, StefanieUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schuermann, SebastianUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Rupitsch, Stefan J.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Heckel, MichaelUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Poeschel, ThorstenUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Winter, LilliUNSPECIFIEDorcid.org/0000-0002-6368-1160UNSPECIFIED
Herrmann, HaraldUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Clemen, Christoph S.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schroeder, RolfUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Friedrich, OliverUNSPECIFIEDorcid.org/0000-0003-2238-2049UNSPECIFIED
URN: urn:nbn:de:hbz:38-134757
DOI: 10.1038/s41598-019-46723-6
Journal or Publication Title: Sci Rep
Volume: 9
Date: 2019
Publisher: NATURE PUBLISHING GROUP
Place of Publication: LONDON
ISSN: 2045-2322
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
VIMENTIN INTERMEDIATE-FILAMENTS; PASSIVE STIFFNESS; MUTANT DESMIN; FORCEMultiple languages
Multidisciplinary SciencesMultiple languages
Refereed: Yes
URI: http://kups.ub.uni-koeln.de/id/eprint/13475

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