MITOCHONRIAL DNA METABOLISM IN YEAST
| FRANCOISE FOURY || Profile |
| || |
Dr. Françoise Foury
- Research Director at the National Fund for Scientific Belgian Research
- Associate Professor at the UCL
Fields of expertise:
- Yeast genetics and molecular biology
- Metabolism of mitochondrial DNA (Polg)
- Biosynthesis of iron-sulfur clusters
Unité de biochimie physiologique
Bt. Carnoy (b.367-10)
5 Croix du Sud (Box 15)
Tel. +32 10 47 46 91
Fax. +32 10 47 38 72
We are using the yeast Saccharomyces cerevisiae as a model organism to understand the molecular bases of two different mitochondrial diseases:
Friedreich ataxia is a recessive progressive neurodegenerative disease, often associated with cardiomyopathy. It is caused by decreased levels of frataxin, a small acidic protein localizing to mitochondria in eukaryotes.
Our group tries to determine the role played by frataxin in the biosynthesis of iron-sulfur clusters, using genetics and molecular biology.
These last five years more than 80 potentially pathogenic alleles of the mitochondrial DNA polymerase (Polg) have been identified in human. They are responsible of rather mild diseases such as external ophtalmoplegia and exercise intolerance, as well as severe neurological disorders leading to strokes, dementia, ataxia and even to a fatal issue in the young child. Polg disorders are associated with multiple deletions or depletion of the mitochondrial DNA.
In order to better understand the molecular bases of the extremely variable clinical presentations caused by Polg mutations, we are constructing yeast mutants bearing mutations that are equivalent to those found in human patients, and we are analyzing in vivo phenotypes and biochemical properties of the purified yeast Polg.
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| Staff 2007-2008 || |
| Group leader || Françoise Foury |
| Ph. D. students || |
Daphné Cochonneau, Sébastien Leidgens, Karolina Szczepanowska
| Staff shared within the whole research unit || |
| Secretary || Monique Leloup |
| Accounting officer || Michèle Rochat |
| Computer officer || Belkacem El Amraoui |
| Engineer || Hervé Degand, Bénédicte Purnelle |
| Technician || Anne-Marie Faber, Régeane Mathieu |
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