ERCC6 and cerebrooculofacioskeletal syndrome 1: Given the broad role of CSB in chromatin remodeling, it has been a major puzzle in the field for why CSB deficiency imposes selective neuronal vulnerability, which has been implicated in multiple rare autosomal recessive syndromes, such as Cockayne Syndrome, DeSanctis-Cacchione syndrome56, UV-Sensitive Syndrome57,58 and Cerebro-Oculo-Facio-Skeletal syndrome 1 (COFS1)59,60.