Grant: Operating Grant

Organelles are membrane-surrounded compartments present within the cells of higher organisms, including humans. Cells contain many functionally distinct organelles that house different, specific and complex biochemical functions that are necessary to the existence of higher life forms. Accordingly, cells have developed mechanisms that ensure the inheritance of their organelles so as to maintain the benefits of having organelles. One essential biological question is how does the cell maintain the population of an organelle during cell division. With every round of cell division, organelles must be duplicated and distributed to daughter cells, while some fraction of the organelles must still be retained in the mother cell. Peroxisomes are organelles involved in fat metabolism and the elimination of toxic chemical substances from the cell. There are inherited diseases called peroxisome biogenesis disorders in which peroxisomes are absent from cells. Patients with a peroxisome biogenesis disorder rarely live beyond the first year. We are investigating how peroxisomes are inherited at cell division and the molecular factors required for this process. Insight into how peroxisomes are inherited will provide increased understanding of the role of organelle inheritance in normal physiology and disease and will further our knowledge of the molecular basis of the peroxisome biogenesis disorders with a view to their future rational treatment.