Sporadic inclusion body myositis (sIBM) is one of the most common myopathies in elderly people. Mitochondrial abnormalities at the histological level are present in these patients. We hypothesize that mitochondrial dysfunction may play a role in disease aetiology. We took the following measurements of muscle and peripheral blood mononuclear cells (PBMCs) from 30 sIBM patients and 38 age- and gender-paired controls: mitochondrial DNA (mtDNA) deletions, amount of mtDNA and mtRNA, mitochondrial protein synthesis, mitochondrial respiratory chain (MRC) complex I and IV enzymatic activity, mitochondrial mass, oxidative stress and mitochondrial dynamics (mitofusin 2 and optic atrophy 1 levels). Depletion of mtDNA was present in muscle from sIBM patients and PBMCs showed deregulated expression of mitochondrial proteins in oxidative phosphorylation. MRC complex IV/citrate synthase activity was significantly decreased in both tissues and mitochondrial dynamics were affected in muscle. Depletion of mtDNA was significantly more severe in patients with mtDNA deletions, which also presented deregulation of mitochondrial fusion proteins. Imbalance in mitochondrial dynamics in muscle was associated with increased mitochondrial genetic disturbances (both depletion and deletions), demonstrating that proper mitochondrial turnover is essential for mitochondrial homoeostasis and muscle function in these patients.
Skip Nav Destination
Article navigation
October 2016
-
Cover Image
Cover Image
Targeted cancer treatment. In this issue, Gopal et al, review the molecular mechanisms of vascular complications of targeted cancer therapies. See pp. 1763–1779 for further details.
Research Article|
August 31 2016
Mitochondrial DNA disturbances and deregulated expression of oxidative phosphorylation and mitochondrial fusion proteins in sporadic inclusion body myositis
Marc Catalán-García;
Marc Catalán-García
*Muscle Research and Mitochondrial Function Laboratory, CELLEX-IDIBAPS, Faculty of Medicine, University of Barcelona; Internal Medicine Department, Hospital Clinic of Barcelona, CIBERER-U722, Barcelona, Spain
Search for other works by this author on:
Glòria Garrabou;
*Muscle Research and Mitochondrial Function Laboratory, CELLEX-IDIBAPS, Faculty of Medicine, University of Barcelona; Internal Medicine Department, Hospital Clinic of Barcelona, CIBERER-U722, Barcelona, Spain
Correspondence: Glòria Garrabou Tornos (email garrabou@clinic.ub.es).
Search for other works by this author on:
Constanza Morén;
Constanza Morén
*Muscle Research and Mitochondrial Function Laboratory, CELLEX-IDIBAPS, Faculty of Medicine, University of Barcelona; Internal Medicine Department, Hospital Clinic of Barcelona, CIBERER-U722, Barcelona, Spain
Search for other works by this author on:
Mariona Guitart-Mampel;
Mariona Guitart-Mampel
*Muscle Research and Mitochondrial Function Laboratory, CELLEX-IDIBAPS, Faculty of Medicine, University of Barcelona; Internal Medicine Department, Hospital Clinic of Barcelona, CIBERER-U722, Barcelona, Spain
Search for other works by this author on:
Adriana Hernando;
Adriana Hernando
*Muscle Research and Mitochondrial Function Laboratory, CELLEX-IDIBAPS, Faculty of Medicine, University of Barcelona; Internal Medicine Department, Hospital Clinic of Barcelona, CIBERER-U722, Barcelona, Spain
Search for other works by this author on:
Àngels Díaz-Ramos;
Àngels Díaz-Ramos
†Institute for Research in Biomedicine (IRB Barcelona), Barcelona Institute of Science and Technology, Barcelona, Spain
‡Departament de Bioquímica i Biomedicina Molecular, Facultat de Biologia, Universitat de Barcelona, 08028 Barcelona, Spain
§CIBER de Diabetes y Enfermedades Metabólicas Asociadas (CIBERDEM), Instituto de Salud Carlos III, Madrid, Spain
Search for other works by this author on:
Ingrid González-Casacuberta;
Ingrid González-Casacuberta
*Muscle Research and Mitochondrial Function Laboratory, CELLEX-IDIBAPS, Faculty of Medicine, University of Barcelona; Internal Medicine Department, Hospital Clinic of Barcelona, CIBERER-U722, Barcelona, Spain
Search for other works by this author on:
Diana-Luz Juárez;
Diana-Luz Juárez
*Muscle Research and Mitochondrial Function Laboratory, CELLEX-IDIBAPS, Faculty of Medicine, University of Barcelona; Internal Medicine Department, Hospital Clinic of Barcelona, CIBERER-U722, Barcelona, Spain
Search for other works by this author on:
Maria Bañó;
Maria Bañó
*Muscle Research and Mitochondrial Function Laboratory, CELLEX-IDIBAPS, Faculty of Medicine, University of Barcelona; Internal Medicine Department, Hospital Clinic of Barcelona, CIBERER-U722, Barcelona, Spain
Search for other works by this author on:
Jennifer Enrich-Bengoa;
Jennifer Enrich-Bengoa
*Muscle Research and Mitochondrial Function Laboratory, CELLEX-IDIBAPS, Faculty of Medicine, University of Barcelona; Internal Medicine Department, Hospital Clinic of Barcelona, CIBERER-U722, Barcelona, Spain
Search for other works by this author on:
Sonia Emperador;
Sonia Emperador
║Departamento de Bioquímica, Biología Molecular y Celular, Universidad de Zaragoza, CIBERER-U727, Instituto de Investigaciones Sanitarias de Aragón, Zaragoza, Spain
Search for other works by this author on:
José César Milisenda;
José César Milisenda
*Muscle Research and Mitochondrial Function Laboratory, CELLEX-IDIBAPS, Faculty of Medicine, University of Barcelona; Internal Medicine Department, Hospital Clinic of Barcelona, CIBERER-U722, Barcelona, Spain
Search for other works by this author on:
Pedro Moreno;
Pedro Moreno
*Muscle Research and Mitochondrial Function Laboratory, CELLEX-IDIBAPS, Faculty of Medicine, University of Barcelona; Internal Medicine Department, Hospital Clinic of Barcelona, CIBERER-U722, Barcelona, Spain
Search for other works by this author on:
Ester Tobías;
Ester Tobías
*Muscle Research and Mitochondrial Function Laboratory, CELLEX-IDIBAPS, Faculty of Medicine, University of Barcelona; Internal Medicine Department, Hospital Clinic of Barcelona, CIBERER-U722, Barcelona, Spain
Search for other works by this author on:
Antonio Zorzano;
Antonio Zorzano
†Institute for Research in Biomedicine (IRB Barcelona), Barcelona Institute of Science and Technology, Barcelona, Spain
‡Departament de Bioquímica i Biomedicina Molecular, Facultat de Biologia, Universitat de Barcelona, 08028 Barcelona, Spain
§CIBER de Diabetes y Enfermedades Metabólicas Asociadas (CIBERDEM), Instituto de Salud Carlos III, Madrid, Spain
Search for other works by this author on:
Julio Montoya;
Julio Montoya
║Departamento de Bioquímica, Biología Molecular y Celular, Universidad de Zaragoza, CIBERER-U727, Instituto de Investigaciones Sanitarias de Aragón, Zaragoza, Spain
Search for other works by this author on:
Francesc Cardellach;
Francesc Cardellach
*Muscle Research and Mitochondrial Function Laboratory, CELLEX-IDIBAPS, Faculty of Medicine, University of Barcelona; Internal Medicine Department, Hospital Clinic of Barcelona, CIBERER-U722, Barcelona, Spain
Search for other works by this author on:
Josep Maria Grau
Josep Maria Grau
*Muscle Research and Mitochondrial Function Laboratory, CELLEX-IDIBAPS, Faculty of Medicine, University of Barcelona; Internal Medicine Department, Hospital Clinic of Barcelona, CIBERER-U722, Barcelona, Spain
Search for other works by this author on:
Publisher: Portland Press Ltd
Received:
February 03 2016
Revision Received:
July 05 2016
Accepted:
July 13 2016
Accepted Manuscript online:
July 13 2016
Online ISSN: 1470-8736
Print ISSN: 0143-5221
© 2016 The Author(s). published by Portland Press Limited on behalf of the Biochemical Society
2016
Clin Sci (Lond) (2016) 130 (19): 1741–1751.
Article history
Received:
February 03 2016
Revision Received:
July 05 2016
Accepted:
July 13 2016
Accepted Manuscript online:
July 13 2016
Citation
Marc Catalán-García, Glòria Garrabou, Constanza Morén, Mariona Guitart-Mampel, Adriana Hernando, Àngels Díaz-Ramos, Ingrid González-Casacuberta, Diana-Luz Juárez, Maria Bañó, Jennifer Enrich-Bengoa, Sonia Emperador, José César Milisenda, Pedro Moreno, Ester Tobías, Antonio Zorzano, Julio Montoya, Francesc Cardellach, Josep Maria Grau; Mitochondrial DNA disturbances and deregulated expression of oxidative phosphorylation and mitochondrial fusion proteins in sporadic inclusion body myositis. Clin Sci (Lond) 1 October 2016; 130 (19): 1741–1751. doi: https://doi.org/10.1042/CS20160080
Download citation file:
Sign in
Don't already have an account? Register
Sign in to your personal account
You could not be signed in. Please check your email address / username and password and try again.
Could not validate captcha. Please try again.