Heteroplasmy of mitochondrial genomes in clonal cultures from patients with Kearns-Sayre syndrome.

Abstract:

:We have analyzed heteroplasmy of mitochondrial DNA in clonal cultures from two patients with Kearns-Sayre syndrome, and have found that individual muscle or fibroblast clones contained either a mixed (i.e. heteroplasmic) population of normal and deleted mitochondrial DNAs, or only normal mitochondrial DNAs (i.e. homoplasmic at a level of detection of less than 1% deleted genomes). The heteroplasmic clones grew significantly more slowly than did "homoplasmic" clones, probably due to defects of respiratory chain enzymes containing mtDNA-encoded polypeptides.

authors

Moraes CT,Schon EA,DiMauro S,Miranda AF

doi

10.1016/0006-291x(89)92499-6

subject

Has Abstract

pub_date

1989-04-28 00:00:00

pages

765-71

issue

2

eissn

0006-291X

issn

1090-2104

pii

0006-291X(89)92499-6

journal_volume

160

pub_type

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