In the paper, published in the journal Nature, researchers from the Patel Group, MRC Weatherall Institute of Molecular Medicine along with colleagues at the MRC Laboratory of Molecular Biology in Cambridge, and Hubrecht Institute in Utrecht, describe a mechanism by which our bodies recognise and respond to toxins and chemotherapy.
Clues to this process came from patients with a rare illness, Cockayne syndrome. Children with Cockayne syndrome develop debilitating symptoms including premature ageing, kidney and brain degeneration, and severe weight loss. The genetic cause of this syndrome, loss of a Cockayne syndrome gene, leaves sufferers unable to repair DNA damage. However, a mouse model of the disease mimicking the loss of a Cockayne gene was unable to recreate the disease profile seen in humans.