Fig. 7

Induction of SMNc gene expression in interferon (IFN)-treated fibroblasts from Spinal muscular atrophy (SMA) individuals. (A) Total RNA from fibroblasts of control (C2) and SMA patients (SMA1, SMA2, SMA3), either untreated (a), treated with IFN-β (1000 U/ml) for 8 hr (b), or treated with IFN-γ (1000 U/ml) for 4 hr (c), were analyzed for SMN and SMNc expression by reverse transcription-polymerase chain reaction (RT-PCR) experiments as described above. (B) RT-PCR experiments were performed using the same RNA samples, but another pair of SMN/SMNc primers, designed to generate a 420-bp and a 360-bp fragment corresponding to the full-lenght transcript of SMN/SMNc and to the alternatively spliced isoform lacking exon 7, respectively. (C) Total protein extracts (100 µg/lane) from fibroblasts of control (C2) and SMA patients (SMA1, SMA2, SMA3), either untreated (a), or treated for 16 hr with IFN-β (1000 U/ml) (b), or with IFN-γ (1000 U/ml) (c) were analyzed by Western blots analysis, as previously described in Fig. 1, using the anti-SMN and anti-actin antibodies.