LTBP4 is important in elastic fibre assembly with LTBP4 mutations resulting in Urban-Rifkin-Davis Syndrome (URDS) also known as autosomal recessive cutis laxa type 1C [134] which is replicated in knockout mouse models [135,136]. This evidence concerns the gene LTBP4 and cutis laxa with severe pulmonary, gastrointestinal and urinary anomalies.