Given that the segmented chromosomes seen in SLF2 and SMC5 patient cells represent a chromosome instability phenotype not previously associated with any known DNA repair or replication deficiency disorder, we have named this syndrome, Atelís Syndrome (ATS), after the Greek word for incomplete to signify the importance of these atelic or segmented chromosomes as a diagnostic marker of the disease. Here, SMC5 is linked to Atelis syndrome.