Description of Research Expertise

Research interests:
Study of iron metabolism, iron trafficking, mitochondrial function, heme and Fe-S proteins.

Key words: mitochondria, yeast, Fe-S, heme, frataxin, ring sideroblast

Research description:
In most eukaryotic cells (including yeast and human cells) the essential iron containing cofactors Fe-S and heme are made in the mitochondria. Since the mitochondrial membrane must be impermeable to ions in order to maintain the membrane potential, the question arises how is iron transported into mitochondria? We have developed methods for measuring iron transport into mitochondria in real time, and we have evidence that specific mitochondrial carrier proteins are involved in the transport process. Genetic, proteomic, and biochemical approaches are being undertaken to learn more about this process - substrate, product, chaperones, regulation and redundancy, perturbations in human diseases.

Rotation Projects for 2006-2007
1. Proteomic project - in collaboration with Dr. Fevzi Daldal. Purification of mitochondria from cell lines and bone marrow cells for characterization of mitochondrial proteome.
2. Synthetic lethal screen with mrs3 mrs4 (mitoferrin orthologs). This is ongoing but not saturated. Mutants in hand already have connections to iron transport, cofactor synthesis, and cell cycle progression.

Lab personnel:
Simon Knight, Ph.D.
Elise Lyver
Heeyong Yoon, Ph.D.
Yan Zhang, Ph.D. (post-doctoral fellow)

Description of Clinical Expertise

diseases of iron metabolism, sideroblastic anemia