Robbesom et al. showed that aberrant fibrillin 1 in the lung is significantly associated with the three most important morphometric parameters of emphysema: alveolar destruction, airspace enlargement and emphysema-related morphological abnormalities; [28] experimental data in mice have confirmed widening of the distal airspaces in Marfan syndrome [29]. Here, FBN1 is linked to Marfan syndrome.