pp 1-25 | Cite as

Doubly Uniparental Inheritance of mtDNA: An Unappreciated Defiance of a General Rule

  • Eleftherios ZourosEmail author
  • George C. Rodakis
Part of the Advances in Anatomy, Embryology and Cell Biology book series


We recount the basic observations about doubly uniparental inheritance (DUI) of mtDNA in bivalvian mollusks with an emphasis on those that were obtained from work in Mytilus and appeared after the review by Zouros (Evol Biol 40:1–31, 2013). Using this information, we present a new model about DUI that is a revised version of previously suggested models. The model can be summarized as follows. A Mytilus female either provides its eggs with the “masculinizing” factor S and the “sperm mitochondria binding” factor Z, or it does not. This property of the female is determined by two nuclear genes, S and Z, that are always in the on/on or the off/off phase. In fertilized eggs without factors S and Z the embryo develops into a female and the sperm mitochondria are randomly dispersed among cells following development. In fertilized eggs with factors S and Z, the first factor causes the cell to become eventually sperm and the second causes the sperm mitochondria to aggregate and anchor to the nuclear membrane by binding to a specific motif of the sperm-derived mtDNA. Factors S and Z are continuously co-synthesized and co-localized in the cell line from the egg to the sperm. The sperm mitochondria of the aggregate escape the mechanism that eliminates the cell’s mitochondria before the formation of the sperm. The rescued mitochondria are subsequently packed into five mega-mitochondria in the sperm and are delivered in the egg.


Blue mussel Doubly uniparental inheritance Mitochondrial DNA inheritance Mytilus 



We thank Dr. M. Ladoukakis for offering helpful comments on an earlier version of the paper.


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© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Department of BiologyUniversity of CreteHeraklionGreece
  2. 2.Department of BiologyNational and Kapodistrian University of AthensAthensGreece

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