Skip to main page content
Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2006 Jan 6;281(1):374-82.
doi: 10.1074/jbc.M509730200. Epub 2005 Nov 1.

Functional Human Mitochondrial DNA Polymerase Gamma Forms a Heterotrimer

Affiliations
Free article

Functional Human Mitochondrial DNA Polymerase Gamma Forms a Heterotrimer

Elena Yakubovskaya et al. J Biol Chem. .
Free article

Abstract

Mitochondrial DNA polymerase gamma (pol gamma) is responsible for replication and repair of mtDNA and is mutated in individuals with genetic disorders such as chronic external ophthalmoplegia and Alpers syndrome. pol gamma is also an adventitious target for toxic side effects of several antiviral compounds, and mutation of its proofreading exonuclease leads to accelerated aging in mouse models. We have used a variety of physical and functional approaches to study the interaction of the human pol gamma catalytic subunit with both the wild-type accessory factor, pol gammaB, and a deletion derivative that is unable to dimerize and consequently is impaired in its ability to stimulate processive DNA synthesis. Our studies clearly showed that the functional human holoenzyme contains two subunits of the processivity factor and one catalytic subunit, thereby forming a heterotrimer. The structure of pol gamma seems to be variable, ranging from a single catalytic subunit in yeast to a heterodimer in Drosophila and a heterotrimer in mammals.

Similar articles

See all similar articles

Cited by 62 articles

See all "Cited by" articles

Publication types

LinkOut - more resources

Feedback