The latent TGFβ binding protein 4 (Ltbp4) locus showed linkage with disease severity in a mouse model of muscular dystrophy[30], and a common haplotype in the human LTBP4 gene, encoding different isoproteins, was found to modify age at loss of ambulation (LoA) in a cohort of patients with severe dystrophinopathy[26]. This evidence concerns the gene LTBP4 and neuromuscular disease caused by qualitative or quantitative defects of dystrophin.