Muscle contractility dysfunction precedes loss of motor unit connectivity in SOD1(G93A) mice.
Muscle Nerve
; 59(2): 254-262, 2019 02.
Article
en En
| MEDLINE
| ID: mdl-30370671
ABSTRACT
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 59254-262, 2019.Palabras clave
Texto completo:
1
Base de datos:
MEDLINE
Asunto principal:
Músculo Esquelético
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Neuronas Motoras
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Contracción Muscular
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Enfermedades Musculares
Tipo de estudio:
Etiology_studies
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Observational_studies
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Prognostic_studies
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Risk_factors_studies
Idioma:
En
Revista:
Muscle Nerve
Año:
2019
Tipo del documento:
Article