Of note, whereas DNA damage has been largely presented as a contributor of SMA pathogenesis, all research has been focused on nucleoplasmic DNA integrity rather than rDNA integrity in SMN-deficient cells (Zhao et al, 2016; Jangi et al, 2017; Kannan et al, 2018, 2020). The gene discussed is SMN2; the disease is proximal spinal muscular atrophy.