Among the subtypes, classical-like EDS (clEDS) is caused by a complete lack of TNX due to homozygous or compound heterozygous TNX gene (TNXB) mutations with autosomal recessive inheritance, leading to nonsense-mediated decay of the mutant RNA (41). This evidence concerns the gene TNXB and Ehlers-Danlos syndrome due to tenascin-X deficiency.