Accumulation of mitochondrial DNA deletions in myotubes cultured from muscles of patients with mitochondrial myopathies.
Mol Gen Genet
; 253(1-2): 182-8, 1996 Nov 27.
Article
em En
| MEDLINE
| ID: mdl-9003302
ABSTRACT
Myoblast cultures were established from muscle biopsies of two patients harboring heteroplasmic mitochondrial (mt) DNA deletions. The accumulation kinetics of the deleted mtDNA was followed during myoblast to myotube differentiation. The percentage of deleted mtDNA was determined by quantitative PCR in myoblasts, myotubes, and muscle biopsies. The deleted form accounted for 65% of the mtDNA present in a muscle biopsy from a patient harboring a 5.6-kb deletion. The percentage of deleted mtDNA was 1.2% in myoblasts and increased progressively after differentiation, up to 12% at 21 days after the commitment time. In a second patient harboring a 2.8-kb deletion, the percentage of deleted mtDNA increased much more slowly from 0.07% in myoblasts to 0.21% after 22 days of differentiation, as compared with 45% in the muscle biopsy. Thus, a three- and ten-fold increase, respectively, in the fraction of deleted mtDNA occurred during the differentiation of myoblasts to myotubes from the two patients. The faster accumulation of deleted mtDNA int he first patient's cells was linked to an earlier myoblast to myotube differentiation, suggesting that the level of deleted mtDNA is inversely related to the rate of cell proliferation.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
DNA Mitocondrial
/
Síndrome de Kearns-Sayre
/
Deleção de Sequência
/
Oftalmoplegia Externa Progressiva Crônica
/
Fibras Musculares Esqueléticas
Limite:
Adolescent
/
Female
/
Humans
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Male
/
Middle aged
Idioma:
En
Revista:
Mol Gen Genet
Ano de publicação:
1996
Tipo de documento:
Article
País de afiliação:
França