Whereas this result in overexpression models using osteosarcoma U2OS and neuroblastoma BE(2)-M17 cells somewhat differs from the cellular fractionation results in patient (fibroblast) cell lines, where nuclear ARH3 protein was unchanged but cytosolic ARH3 was lost, it demonstrates that dysregulation of the subcellular localization of ARH3(V335G)-GFP is due to the mutation rather than loss of catalytic activity (Fig 3). The gene discussed is ADPRS; the disease is osteosarcoma.