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

Institut for Klinisk Medicin

4 Citationer (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.

Originalsprog	Engelsk
Tidsskrift	Mitochondrion
Vol/bind	13
Udgave nummer	2
Sider (fra-til)	63-70
Antal sider	8
ISSN	1567-7249
DOI	https://doi.org/10.1016/j.mito.2013.01.009
Status	Udgivet - mar. 2013

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10.1016/j.mito.2013.01.009

Citationsformater

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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.",

author = "Krag, {T O} and S Hauerslev and Jeppesen, {T D} and M Duno and J Vissing",

year = "2013",

month = mar,

doi = "10.1016/j.mito.2013.01.009",

language = "English",

volume = "13",

pages = "63--70",

journal = "Mitochondrion",

issn = "1567-7249",

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TY - JOUR

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

C2 - 23376095

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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