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 S, Heckel M, Pöschel T, Winter L, Herrmann H, Clemen CS, Schröder R, Friedrich O (2019)

Publication Type: Journal article

Publication year: 2019

Journal

Scientific Reports Nature Publishing Group: Open Access Journals - Option B

Book Volume: 9

Article Number: 10769

Journal Issue: 1

DOI: 10.1038/s41598-019-46723-6

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.

Authors with CRIS profile

Michael Haug Lehrstuhl für Medizinische Biotechnologie Charlotte Meyer Lehrstuhl für Medizinische Biotechnologie Barbara Reischl Lehrstuhl für Medizinische Biotechnologie Gerhard Prölß Lehrstuhl für Medizinische Biotechnologie Karoline Menacher Lehrstuhl für Kunststofftechnik Julian Iberl Lehrstuhl für Prozessmaschinen und Anlagentechnik Stefanie Nübler Lehrstuhl für Medizinische Biotechnologie Sebastian Schürmann Lehrstuhl für Medizinische Biotechnologie Stefan Rupitsch Lehrstuhl für Autonome Systeme und Mechatronik Michael Heckel Lehrstuhl für Multiscale Simulation of Particulate Systems Thorsten Pöschel Lehrstuhl für Multiscale Simulation of Particulate Systems Rolf Schröder Professur für Neuropathologie Oliver Friedrich Lehrstuhl für Medizinische Biotechnologie

Involved external institutions

Berufsgenossenschaftliches Universitätsklinikum Bergmannsheil DE Germany (DE)

How to cite

APA:

Haug, M., Meyer, C., Reischl, B., Prölß, G., Vetter, K., Iberl, J.,... Friedrich, O. (2019). The MyoRobot technology discloses a premature biomechanical decay of skeletal muscle fiber bundles derived from R349P desminopathy mice. Scientific Reports, 9(1). https://doi.org/10.1038/s41598-019-46723-6

MLA:

Haug, Michael, et al. "The MyoRobot technology discloses a premature biomechanical decay of skeletal muscle fiber bundles derived from R349P desminopathy mice." Scientific Reports 9.1 (2019).

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