LTBP4 and pulmonary emphysema: Genetic deletion targeting both Ltbp4 isoforms in mice produces severe neonatal lethality [11], while mice lacking the short Ltbp4 (Ltbp4S−/− mice) replicate the major human phenotype with pulmonary emphysema [11] and, thus, represents a widely accepted animal platform for understanding the pathogenesis of dysregulated LTBP4/TGFβ signalling.