In contrast, the relative amount of PTC-containing FBN1 transcripts in blood was found to be significantly higher than in affected fibroblasts with experimental inhibition of nonsense-mediated decay, while in fibroblasts without NMD inhibition, no mutant alleles could be detected at all (Magyar et al., 2009), implying that tissue-specific degradation of transcripts also plays an important role in MFS pathogenesis. Here, FBN1 is linked to Marfan syndrome.