Ataxia-Telangiectasia: An emerging role for inflammation in driving neurodegeneration and premature ageing

Understanding how genomic instability leads to the transcriptional misregulation of genes central to neuronal homeostasis is critical for understanding how persistent DNA damage drives A-T pathology. This project seeks to advance understanding of the relationship between DNA damage, its transcriptional consequences and cellular inflammation that triggers senescence by combining cutting-edge technologies (DNA combing, BLISS, spatial transcriptomics) with our novel human A-T patient stem cell-derived brain organoid model to test the novel concept that senostatics targeting the cGAS and STING pathway can slow neurodegeneration in A-T.