Next we examined the effect of UPR activation following readthrough treatment in another disease model, a human fibroblast cell line (P133) derived from a patient with immunodeficiency with microcephaly, homozygous for the nonsense mutation R178X in the Cernunnos/XRCC4-like factor (XLF) gene (Buck et al, 2006). The gene discussed is NHEJ1; the disease is immunodeficiency disease.