MRC Mitochondrial Biology Unit

The MRC-MBU is on a serious mission to understand mitochondrial biology in health and disease, and to exploit this understanding to develop new therapies and improve human health.

Destroying mutant mitochondrial DNA!

MRC Mitochondrial Biology Unit, University of Cambridge is destroying Mutant Mitochondrial DNA!  Our aim is to destroy mutant (bad) mitochondrial DNA by fishing it out of the mitochondrion and placing it in the mtDNA dustbin. Check out their YouTube video of this fun activity available at RAREfest22.

DNA is the instruction manual for cells; the sequence of special chemicals called bases, which are either A, T, C and G, determines the sequence of amino acids in a protein, and protein is what makes us! Mitochondria are the power stations of the cell, providing us with energy. They have many copies of a circular molecule of DNA, known as mitochondrial DNA, which contain the instructions to make some important proteins that are found within mitochondria. A mutation is when one base is replaced by another by mistake (for example, a T might be replaced by a G). This could change the sequence of amino acids in a protein, which could mean that the protein no longer works properly. This may cause disease.

As mitochondria contain many copies of mitochondrial DNA, there is often a mixture of normal (good) DNA and mutated (bad) DNA. How severe a disease is often dependent on the ratio of good DNA to bad DNA. If there is more good DNA than bad, the disease will be mild; if there is more bad DNA than good, the disease may be severe.

We have made a chemical, called mtZFN, that can destroy only the mutated (bad) DNA, leaving the normal (good) DNA behind. The good DNA can then copy itself, and the mitochondria can function as normal!

View the poster about the MRC Mitochondrial Biology Unit’s activity at RAREfest22.

MRC Mitochondrial Biology Unit, University of Cambridge is destroying Mutant Mitochondrial DNA! Come mutant DNA fishing with us and beat the clock!