Review
Maternal inheritance of mitochondrial DNA by diverse mechanisms to eliminate paternal mitochondrial DNA

https://doi.org/10.1016/j.bbamcr.2013.03.010Get rights and content
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Highlights

  • Mitochondrial DNA is inherited maternally in most animals.

  • There are diverse mechanisms to eliminate paternal mitochondria or their DNA in zygotes.

  • Paternal mitochondria are degraded by autophagy in C. elegans or the ubiquitin–mediated mechanism in mammals.

  • Paternal mtDNA is degraded by specific nucleases in some species.

  • Paternal mitochondria and/or their mtDNA are physically excluded prior or on fertilization in some species.

Abstract

The mitochondrion is an organelle that has its own DNA (mtDNA). Mitochondria play essential roles in energy production and in various cellular processes such as metabolism and signal transduction. In most animals, including humans, although the sperm-derived paternal mitochondria enter the oocyte cytoplasm after fertilization, their mtDNA is never transmitted to the offspring. This pattern of mtDNA inheritance is well known as “maternal inheritance.” However, how the paternal mitochondria and mtDNA are eliminated from the cytoplasm of gametes or zygotes remains an enigma. Recently, a variety of mechanisms, including specific nuclease-dependent systems, ubiquitin–proteasome system, and autophagy have been shown to degrade the paternal mtDNA or the paternal mitochondria themselves in order to prevent paternal mtDNA transmission. In this review, we will address the current state of knowledge of the molecular mechanisms underlying the elimination of paternal mtDNA or mitochondrial structures for ensuring the maternal transmission of mtDNA.

Keywords

Mitochondria
Mitochondrial DNA
Maternal inheritance
Fertilization
Ubiquitin–proteasome system
Autophagy

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