It remains unknown whether Xbp1 splicing, Atf6 proteolysis, or Eif2α phosphorylation occur in other mouse models of skeletal dysplasias characterized by ER stress, including the p.V194D Matn3 mutant model of multiple epiphyseal dysplasia [39], and an Aga2-mutant model of osteogenesis imperfecta [40]. This evidence concerns the gene ATF6 and skeletal dysplasia.