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Mitochondrial DNA Inheritance

by Georgina Cornwall, PhD
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      Slides 03 Expansions and Mitochondrial Inheritance SingleGeneDisorders.pdf
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    The next piece that we need to move into is mitochondrial inheritance. As I’m sure you know, mitochondria have DNA and so we have to talk about how it’s inherited. You probably already know that the mitochondria are inherited from the female parent. On mitochondrial DNA, there are 37 different genes that can encode enzymes that are involved in oxidative phosphorylation. We know that the mitochondria are the powerhouse, right? Glycolysis, Krebs cycle, electron transport chain, all of that happens around the mitochondria as we produce ATP. Oxidative phosphorylation was that electron transport chain piece that happens within the mitochondria. We have RNA in there that is necessary for translating and making all of the enzymes involved in that process. Mitochondria then come from the egg. You can see here a nice graphic showing mitochondria, the little red dots inside our egg cell and the sperm swimming along. You probably recall that mitochondria are not included inside the head of the sperm. But he does need lots of energy to do his job and swim to his target. He does have mitochondria but they’re all sort of outside of the nuclear region and don’t make their way into the egg. Mitochondria come from an internal line of inheritance. We will not see any passage of mitochondrial genes from a male to his offspring. Here’s a pedigree showing mitochondrial inheritance of a disorder you should probably know about also which is Leber hereditary optic neuropathy which is early onset degenerative eye disorder, so loss of sight. It is clearly inherited by mitochondrial DNA. You can recognize this in the pedigree when you see the father that’s affected does not pass it on to any of his offspring, whereas a mother that is affected passes it on to most offspring. Now, why...

    About the Lecture

    The lecture Mitochondrial DNA Inheritance by Georgina Cornwall, PhD is from the course Single-Gene Disorders.


    Author of lecture Mitochondrial DNA Inheritance

     Georgina Cornwall, PhD

    Georgina Cornwall, PhD


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