Mid-Term Report Summary - MITOPEXLYSONETWORK (Mitochondria, Peroxisomes and Lysosomes - the "menage a trois" of cellular metabolism)
Cells contain several specialized structures called organelles. Each organelle has specific functions inside the cell. Several organelles are involved in metabolism of sugar, amino acids and fatty acids, of which mitochondria, lysosomes and peroxisomes are the most relevant examples. It has been known for a couple of decades that mutations in genes which encode for proteins in mitochondria, peroxisomes or lysosomes results in very severe diseases, often leading to death within the first year of age. Some forms of these diseases manifest in older children and young adults, are highly debilitating and result in loss of quality of life and premature death. Currently the molecular mechanisms of these diseases are not clearly understood, and the therapeutic approach to them consists in the management of symptoms rather than a treatment of the cause. Furthermore, the diseases of mitochondria, peroxisomes and lysosomes have been addressed as diseases of a single organelle. Our research shows that genetic perturbations of mitochondria, peroxisomes or lysosomes result in secondary perturbations to the other two organelles and other cellular structures. Furthermore, we have found signaling pathways that respond to perturbations in these organelles, and some of them are common to defects in different organelles.
Christiane Hennecke, (Director International Relations)
Record Number: 189658 / Last updated on: 2016-10-13