Muscle contractility dysfunction precedes loss of motor unit connectivity in SOD1(G93A) mice.

Autor: Wier CG; Department of Biological Chemistry and Pharmacology, The Ohio State University Wexner Medical Center, Columbus, Ohio, USA., Crum AE; Department of Neurology, Division of Neuromuscular Medicine, The Ohio State University Wexner Medical Center, 395 West 12th Avenue, Columbus, Ohio, 43210, USA., Reynolds AB; Department of Biological Chemistry and Pharmacology, The Ohio State University Wexner Medical Center, Columbus, Ohio, USA., Iyer CC; Department of Neurology, Division of Neuromuscular Medicine, The Ohio State University Wexner Medical Center, 395 West 12th Avenue, Columbus, Ohio, 43210, USA., Chugh D; Department of Neurology, Division of Neuromuscular Medicine, The Ohio State University Wexner Medical Center, 395 West 12th Avenue, Columbus, Ohio, 43210, USA., Palettas MS; Center for Biostatistics, Department of Biomedical Informatics, The Ohio State University, Columbus, Ohio, USA., Heilman PL; Department of Neurology, Division of Neuromuscular Medicine, The Ohio State University Wexner Medical Center, 395 West 12th Avenue, Columbus, Ohio, 43210, USA., Kline DM; Center for Biostatistics, Department of Biomedical Informatics, The Ohio State University, Columbus, Ohio, USA., Arnold WD; Department of Neurology, Division of Neuromuscular Medicine, The Ohio State University Wexner Medical Center, 395 West 12th Avenue, Columbus, Ohio, 43210, USA.; Department of Physical Medicine and Rehabilitation, The Ohio State University Wexner Medical Center, Columbus, Ohio, USA.; Department of Neuroscience, The Ohio State University Wexner Medical Center, Columbus, Ohio, USA.; Department of Physiology and Cell Biology, The Ohio State University Wexner Medical Center, Columbus, Ohio, USA., Kolb SJ; Department of Biological Chemistry and Pharmacology, The Ohio State University Wexner Medical Center, Columbus, Ohio, USA.; Department of Neurology, Division of Neuromuscular Medicine, The Ohio State University Wexner Medical Center, 395 West 12th Avenue, Columbus, Ohio, 43210, USA.
Jazyk: angličtina
Zdroj: Muscle & nerve [Muscle Nerve] 2019 Feb; Vol. 59 (2), pp. 254-262. Date of Electronic Publication: 2018 Dec 21.
DOI: 10.1002/mus.26365
Abstrakt: Introduction: Electrophysiological measurements are used in longitudinal clinical studies to provide insight into the progression of amyotrophic lateral sclerosis (ALS) and the relationship between muscle weakness and motor unit (MU) degeneration. Here, we used a similar longitudinal approach in the Cu/Zn superoxide dismutase (SOD1[G93A]) mouse model of ALS.
Methods: In vivo muscle contractility and MU connectivity assays were assessed longitudinally in SOD1(G93A) and wild type mice from postnatal days 35 to 119.
Results: In SOD1(G93A) males, muscle contractility was reduced by day 35 and preceded MU loss. Muscle contractility and motor unit reduction were delayed in SOD1(G93A) females compared with males, but, just as with males, muscle contractility reduction preceded MU loss.
Discussion: The longitudinal contractility and connectivity paradigm employed here provides additional insight into the SOD1(G93A) mouse model and suggests that loss of muscle contractility is an early finding that may precede loss of MUs and motor neuron death. Muscle Nerve 59:254-262, 2019.
(© 2018 Wiley Periodicals, Inc.)
Databáze: MEDLINE
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