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Amyotrophic Lateral Sclerosis and swim training affect copper metabolism in skeletal muscle in a mouse model of disease.
Bialobrodzka, Emilia; Flis, Damian Jozef; Akdogan, Banu; Borkowska, Andzelika; Wieckowski, Mariusz Roman; Antosiewicz, Jedrzej; Zischka, Hans; Dzik, Katarzyna Patrycja; Kaczor, Jan Jacek; Ziolkowski, Wieslaw.
Afiliación
  • Bialobrodzka E; Poznan University of Physical Education, Poznan, Poland.
  • Flis DJ; Department of Pharmaceutical Pathophysiology, Faculty of Pharmacy, Medical University of Gdansk, Gdansk, Poland.
  • Akdogan B; Institute of Molecular Toxicology and Pharmacology, Helmholtz Center Munich, German Research Center for Environmental Health, Neuherberg, Germany.
  • Borkowska A; Department of Bioenergetics and Physiology of Exercise, Faculty of Health Sciences, Medical University of Gdansk, Gdansk, Poland.
  • Wieckowski MR; Laboratory of Mitochondrial Biology and Metabolism, Nencki Institute of Experimental Biology, Warsaw, Poland.
  • Antosiewicz J; Department of Bioenergetics and Physiology of Exercise, Faculty of Health Sciences, Medical University of Gdansk, Gdansk, Poland.
  • Zischka H; Institute of Molecular Toxicology and Pharmacology, Helmholtz Center Munich, German Research Center for Environmental Health, Neuherberg, Germany.
  • Dzik KP; Institute of Toxicology and Environmental Hygiene, Technical University Munich School of Medicine, Munich, Germany.
  • Kaczor JJ; Department of Animal and Human Physiology, University of Gdansk, Gdansk, Poland.
  • Ziolkowski W; Department of Animal and Human Physiology, University of Gdansk, Gdansk, Poland.
Muscle Nerve ; 70(5): 1111-1118, 2024 Nov.
Article en En | MEDLINE | ID: mdl-39225106
ABSTRACT
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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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Natación / Ratones Transgénicos / Músculo Esquelético / Cobre / Modelos Animales de Enfermedad / Esclerosis Amiotrófica Lateral Límite: Animals Idioma: En Revista: Muscle Nerve Año: 2024 Tipo del documento: Article País de afiliación: Polonia Pais de publicación: Estados Unidos
Texto completo
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XML
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Natación / Ratones Transgénicos / Músculo Esquelético / Cobre / Modelos Animales de Enfermedad / Esclerosis Amiotrófica Lateral Límite: Animals Idioma: En Revista: Muscle Nerve Año: 2024 Tipo del documento: Article País de afiliación: Polonia Pais de publicación: Estados Unidos