Cockayne Syndrome and beyond- do we find the pathophysiology of premature aging in aging-associated disease?

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Cockayne Syndrome is a rare model-disease, whose investigation should yield deeper insights into the mechanisms of aging. With the proposed works we want to show that the disease-mechanisms we already identified are relevant for the understanding of common aging-associated diseases like Huntington and Alzheimers. Moreover, cell culture experiments should pave the way for first clinical trials to test if pharmaceutical drugs can improve the disturbed protein synthesis in Cockayne syndrome children. In depth analysis of the protein synthesis machinery should identify the underlying defects and elucidate protein synthesis mechanisms of the aging body. As dysbalances in proteinhomeostasis are common symptoms of aging-associated diseases, we want to prove the hypothesis that a disturbed protein synthesis might play a central role in these pathomechanisms. The proposed works intend to lead to a new assessment of the hitherto underestimated process of protein synthesis in aging.