Muscle regeneration in mitochondrial myopathies

T O Krag; S Hauerslev; T D Jeppesen; M Duno; J Vissing

doi:10.1016/j.mito.2013.01.009

Muscle regeneration in mitochondrial myopathies

T O Krag, S Hauerslev, T D Jeppesen, M Duno, J Vissing

Department of Clinical Medicine

4 Citations (Scopus)

Abstract

Mitochondrial myopathies cover a diverse group of disorders in which ragged red and COX-negative fibers are common findings on muscle morphology. In contrast, muscle degeneration and regeneration, typically found in muscular dystrophies, are not considered characteristic features of mitochondrial myopathies. We investigated regeneration in muscle biopsies from 61 genetically well-defined patients affected by mitochondrial myopathy. Our results show that the perturbed energy metabolism in mitochondrial myopathies causes ongoing muscle regeneration in a majority of patients, and some were even affected by a dystrophic morphology. The results add to the complexity of the pathogenesis underlying mitochondrial myopathies, and expand the knowledge about the impact of energy deficiency on another aspect of muscle structure and function.

Original language	English
Journal	Mitochondrion
Volume	13
Issue number	2
Pages (from-to)	63-70
Number of pages	8
ISSN	1567-7249
DOIs	https://doi.org/10.1016/j.mito.2013.01.009
Publication status	Published - Mar 2013

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title = "Muscle regeneration in mitochondrial myopathies",

abstract = "Mitochondrial myopathies cover a diverse group of disorders in which ragged red and COX-negative fibers are common findings on muscle morphology. In contrast, muscle degeneration and regeneration, typically found in muscular dystrophies, are not considered characteristic features of mitochondrial myopathies. We investigated regeneration in muscle biopsies from 61 genetically well-defined patients affected by mitochondrial myopathy. Our results show that the perturbed energy metabolism in mitochondrial myopathies causes ongoing muscle regeneration in a majority of patients, and some were even affected by a dystrophic morphology. The results add to the complexity of the pathogenesis underlying mitochondrial myopathies, and expand the knowledge about the impact of energy deficiency on another aspect of muscle structure and function.",

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T1 - Muscle regeneration in mitochondrial myopathies

AU - Krag, T O

AU - Hauerslev, S

AU - Jeppesen, T D

AU - Duno, M

AU - Vissing, J

PY - 2013/3

Y1 - 2013/3

N2 - Mitochondrial myopathies cover a diverse group of disorders in which ragged red and COX-negative fibers are common findings on muscle morphology. In contrast, muscle degeneration and regeneration, typically found in muscular dystrophies, are not considered characteristic features of mitochondrial myopathies. We investigated regeneration in muscle biopsies from 61 genetically well-defined patients affected by mitochondrial myopathy. Our results show that the perturbed energy metabolism in mitochondrial myopathies causes ongoing muscle regeneration in a majority of patients, and some were even affected by a dystrophic morphology. The results add to the complexity of the pathogenesis underlying mitochondrial myopathies, and expand the knowledge about the impact of energy deficiency on another aspect of muscle structure and function.

AB - Mitochondrial myopathies cover a diverse group of disorders in which ragged red and COX-negative fibers are common findings on muscle morphology. In contrast, muscle degeneration and regeneration, typically found in muscular dystrophies, are not considered characteristic features of mitochondrial myopathies. We investigated regeneration in muscle biopsies from 61 genetically well-defined patients affected by mitochondrial myopathy. Our results show that the perturbed energy metabolism in mitochondrial myopathies causes ongoing muscle regeneration in a majority of patients, and some were even affected by a dystrophic morphology. The results add to the complexity of the pathogenesis underlying mitochondrial myopathies, and expand the knowledge about the impact of energy deficiency on another aspect of muscle structure and function.

U2 - 10.1016/j.mito.2013.01.009

DO - 10.1016/j.mito.2013.01.009

M3 - Journal article

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SN - 1567-7249

VL - 13

SP - 63

EP - 70

JO - Mitochondrion

JF - Mitochondrion

IS - 2

ER -

Muscle regeneration in mitochondrial myopathies

Abstract

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