Cutting-Edge Technology and Mitochondrial Diseases - Where is the Limit?

In their latest study, published in the Nature, Shoukrat Mitalipov and collaborators, including Juan Carlos Izpisua Belmonte, reported on two potential 'gene correction' strategies that can help patients with mitochondrial diseases (1, BioNews 811). Both approaches are built on the idea of mitochondria segregation phenomenon, reported for the first time by a group from the Mayo clinic in Rochester, Minnesota(2).

Basically, in proliferating cells the mitochondria segregate spontaneously and, if we start with a heteroplasmic cell containing a mix of healthy, wild-type and mutation-carrying mitochondria, after multiple cell divisions daughter cells will segregate into three major groups. The first will contain predominantly healthy, wild-type mitochondria with a few-to-nil mitochondria that carry the mutation, while the second is quite opposite – a vast majority of mitochondria will be carrying the deleterious mutation and almost none will be healthy. The third group will be heteroplasmic, containing various degrees of mixed normal and mutation-carrying mitochondria.

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