Dataset Information

Healthy, mtDNA-mutation free mesoangioblasts from mtDNA patients qualify for autologous therapy.

ABSTRACT:

Background

Myopathy and exercise intolerance are prominent clinical features in carriers of a point-mutation or large-scale deletion in the mitochondrial DNA (mtDNA). In the majority of patients, the mtDNA mutation is heteroplasmic with varying mutation loads between tissues of an individual. Exercise-induced muscle regeneration has been shown to be beneficial in some mtDNA mutation carriers, but is often not feasible for this patient group. In this study, we performed in vitro analysis of mesoangioblasts from mtDNA mutation carriers to assess their potential to be used as source for autologous myogenic cell therapy.

Methods

We assessed the heteroplasmy level of patient-derived mesoangioblasts, isolated from skeletal muscle of multiple carriers of different mtDNA point-mutations (n?=?25). Mesoangioblast cultures with ResultsThis study demonstrated that mesoangioblasts in half of the patients contained no or a very low mutation load (ConclusionsOur data demonstrates that in half of the mtDNA mutation carriers, their mesoangioblasts are (nearly) mutation free and can potentially be used as source for autologous cell therapy for generation of new muscle fibers without mtDNA mutation and normal mitochondrial function.

SUBMITTER: van Tienen F

PROVIDER: S-EPMC6925445 | biostudies-literature | 2019 Dec

REPOSITORIES: biostudies-literature

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Healthy, mtDNA-mutation free mesoangioblasts from mtDNA patients qualify for autologous therapy.

van Tienen Florence F Zelissen Ruby R Timmer Erika E van Gisbergen Marike M Lindsey Patrick P Quattrocelli Mattia M Sampaolesi Maurilio M Mulder-den Hartog Elvira E de Coo Irenaeus I Smeets Hubert H

Stem cell research & therapy 20191221 1

<h4>Background</h4>Myopathy and exercise intolerance are prominent clinical features in carriers of a point-mutation or large-scale deletion in the mitochondrial DNA (mtDNA). In the majority of patients, the mtDNA mutation is heteroplasmic with varying mutation loads between tissues of an individual. Exercise-induced muscle regeneration has been shown to be beneficial in some mtDNA mutation carriers, but is often not feasible for this patient group. In this study, we performed in vitro analysis ...[more]

PMID: 31864395

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Dataset Information

Healthy, mtDNA-mutation free mesoangioblasts from mtDNA patients qualify for autologous therapy.

Background

Methods

Publications

Healthy, mtDNA-mutation free mesoangioblasts from mtDNA patients qualify for autologous therapy.

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