LTBP4 and cutis laxa with severe pulmonary, gastrointestinal and urinary anomalies: Mutations in LTBP4 are associated with an inherited connective tissue disease, autosomal recessive cutis laxa type 1C (ARCLIC) in humans [55], which is recapitulated by an ARCLIC-like phenotype in LTBP4 deficient mice [48].