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What Is Kearns-Sayre Syndrome After All?
Arch Neurol. 2001;58:1053-1054.
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| Since this article does not have an abstract, we have provided the first 150 words of the full text and any section headings. |
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EACH MITOCHONDRION contains multiple copies of its own DNA (mtDNA),
and possesses its own replication, transcription, and translation machineries.1 Each cell has thousands of copies of mtDNA molecules
unlike the nuclear genome. Human mtDNA encodes 13 polypeptides of the respiratory
chain (complexes I, III, IV, and V) subunits, 28 ribosomal RNAs, and 22 transfer
RNAs (tRNAs), in a circular double-stranded genome of approximately 16.5 kilobases.2 Each of the 13 polypeptide-coding genes allows production
of a single subunit of the respiratory chain, whereas the tRNA genes are involved
in the synthesis of many respiratory chain components. While these mitochondrial
gene products are used within the mitochondrion, they are insufficient to
support the full function of the mitochondrion. A significant part of mitochondrial
proteins are, indeed, coded by the nuclear genes. Furthermore, the endosymbiosis
between mitochondria and the host cell is balanced on intricate interactions
among genes and gene products coded by . . . [Full Text of this Article]
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ABSTRACT
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