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Brain 2006 129(7):1637-1639; doi:10.1093/brain/awl169
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© The Author (2006). Published by Oxford University Press on behalf of the Guarantors of Brain. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

Scientific Commentary

A polymorphic polymerase

The first 10% of the full text of this article appears below.

As implied by Greek etymology, polymerases make multiple (poly) identical parts (meroi) of a template. Specifically, polymerase {gamma} (POLG) is in charge of replicating the mitochondrial DNA (mtDNA) faithfully and steadily, so that mitochondria have adequate numbers of mtDNAs to maintain the structure and function of the respiratory chain. Two other terms with the same root apply to POLG: polymeric and polymorphic.

Polymeric refers to the fact that POLG is made of three subunits, one catalytic subunit, POLG proper, with polymerase (i.e. replicating) and exonuclease (i.e. proofreading) activities, and two smaller identical accessory subunits, POLG2, which are responsible for processive DNA synthesis and tight binding of the POLG complex to DNA (Yakubovskaya et al., 2006Go).

Polymorphic refers to the extraordinary spectrum of clinical phenotypes that have been associated with mutations in the POLG gene and are the subject of two papers in this issue of Brain. . . . [Full Text of this Article]

Salvatore DiMauro, Guido Davidzon and Michio Hirano

Department of Neurology, Columbia University Medical Center New York, NY, USA

E-mail: sd12@columbia.edu


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