We suggest that reduced IdoA content in CS/DS chains in the notochord and sclerotome not only relates to the kinked tail phenotype in Dse-morphant Xenopus tadpoles [16] as well as Dse-KO and DKO mouse embryos and pups [12–15], but also explains the spinal and chest wall deformities in MS-EDS patients [46]. Here, DSE is linked to Ehlers-Danlos syndrome.