Dermal fibroblasts from patient II:3 and three healthy controls were obtained by standard methods and cultured in Dulbecco’s Modified Eagle’s Medium supplemented by 10% foetal calf serum and 1% penicillin/streptomycin (DMEM-10). Human recombinant IFNα2b (Intron-A, Schering-Plough, USA) and IFNγ (Immunikin, Boehringer Ingelheim, Germany) were used at 1000 IU/ml. Immunoblotting was carried out as previously described (20 (link)). Antibodies used are described in Table 1
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