Tissue distribution and transmission of mitochondrial DNA deletions in mitochondrial myopathies.

Zeviani, M; Gellera, C; Pannacci, M; Uziel, G; Prelle, A; Servidei, S; DiDonato, S · Ann Neurol · 1990

basic_science · Level V

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Abstract

By using a combination of Southern blot hybridization analysis, polymerase-chain reaction amplification, and direct nucleotide sequencing, we studied deletions of mitochondrial DNA (mtDNA) in several nonfamilial patients with progressive external ophthalmoplegia and Kearns-Sayre syndrome, and in some of their direct relatives. Results suggest that the heteroplasmic mtDNA populations are already present at a very early stage of development, and that there is no direct transmission of mtDNA heteroplasmy by maternal inheritance.

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