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Białobrodzka, E.* ; Flis, D.J.* ; Akdogan, B. ; Borkowska, A.* ; Wieckowski, M.R.* ; Antosiewicz, J.* ; Zischka, H. ; Dzik, K.P.* ; Kaczor, J.J.* ; Ziolkowski, W.*

Amyotrophic Lateral Sclerosis and swim training affect copper metabolism in skeletal muscle in a mouse model of disease.

Muscle Nerve 70, 1111-1118 (2024)
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INTRODUCTION/AIMS: Swim training and regulation of copper metabolism result in clinical benefits in amyotrophic lateral sclerosis (ALS) mice. Therefore, the study aimed to determine whether swim training improves copper metabolism by modifying copper metabolism in the skeletal muscles of ALS mice. METHODS: SOD1G93A mice (n = 6 per group) were used as the ALS model, and wild-type B6SJL (WT) mice as controls (n = 6). Mice with ALS were analyzed before the onset of ALS (ALS BEFORE), at baseline ALS (first disease symptoms, trained and untrained, ALS ONSET), and at the end of ALS (last stage disease, trained and untrained, ALS TERMINAL). Copper concentrations and the level of copper metabolism proteins in the skeletal muscles of the lower leg were determined. RESULTS: ALS disease caused a reduction in the copper concentration in ALS TERMINAL untrained mice compared with the ALS BEFORE (10.43 ± 1.81 and 38.67 ± 11.50 μg/mg, respectively, p = .0213). The copper chaperon for SOD1 protein, which supplies copper to SOD1, and ATPase7a protein (copper exporter), increased at the terminal stage of disease by 57% (p = .0021) and 34% (p = .0372), while the CTR1 protein (copper importer) decreased by 45% (p = .002). Swim training moderately affected the copper concentration and the concentrations of proteins responsible for copper metabolism in skeletal muscles. DISCUSSION: The results show disturbances in skeletal muscle copper metabolism associated with ALS progression, which is moderately affected by swim training. From a clinical point of view, exercise in water for ALS patients should be an essential element of rehabilitation for maintaining quality of life.
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Publication type Article: Journal article
Document type Scientific Article
Corresponding Author
Keywords Als ; Copper Metabolism ; Copper Transport ; Exercise ; Neurodegeneration; Motor-neuron Disease; Sod1; Mice; Overexpression; Dysregulation; Survival; Ccs; Sex
ISSN (print) / ISBN 0148-639X
e-ISSN 1097-4598
Journal Muscle & Nerve
Quellenangaben Volume: 70, Issue: 5, Pages: 1111-1118 Article Number: , Supplement: ,
Publisher Wiley
Publishing Place 111 River St, Hoboken 07030-5774, Nj Usa
Non-patent literature Publications
Reviewing status Peer reviewed
Institute(s) Institute of Molecular Toxicology and Pharmacology (TOXI)
Grants Narodowe Centrum Nauki