As mutations in SMARCAL1 cause the multisystemic genetic disease SIOD (Boerkoel et al., 2002) and complete loss of function of SMARCAL1 correlates with the severest form of this condition (Elizondo et al., 2009), our conditional knockdown of SMARCAL1 in iPSCs may represent a powerful model for studying SIOD pathogenetic mechanisms and complement other model systems in recapitulating the disease. The gene discussed is SMARCAL1; the disease is Schimke immuno-osseous dysplasia.