Ribosomes are large macromolecular machines that catalyze protein synthesis in all cells. Ribosome assembly constitutes perhaps the major metabolic activity of a growing cell, to ensure its protein synthesis capacity. Because ribosome assembly is so energy costly and only desired when cells are growing, ribosome assembly is highly regulated in response to available nutrients and external stimuli. Conversely, rapidly growing cells, such as cancer cells, have developed mechanisms to upregulate ribosome assembly.
In addition to producing enough ribosomes, cells must also ensure that the ribosomes they make are fully functional. Failure to do so is the cause of several human diseases including Diamond Blackfan Anemia, 5q- syndrome, congenital asplenia and many others. Surprisingly, these diseases are characterized not only by the growth defects expected from a lack of functional ribosomes, but also by a highly increased risk of cancer.
In our lab we study the mechanisms that cells use to regulate and quality control the assembly of ribosomes, with the goal of understanding the molecular basis for cancer when ribosomes are defective and of developing novel drug targets for cancer therapy.
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