FBN1 and systemic sclerosis: As already mentioned, Tsk/+ mice replicate some MFS features (bone lengthening, cardiac hypertrophy and lung emphysema) as well as characteristic SSc features (skin fibrosis with excessive accumulation of collagen I and microfibrils, and the emergence of circulating autoantibodies against topoisomerase I, RNA polymerase I and fibrillin-1) [71-74].