Biallelic mutations in TNXB are associated with classical-like Ehlers-Danlos syndrome, and share many symptoms with COL6-RD.[27–30] COMP (cartilage oligomeric matrix protein) is another thrombospondin family gene (thrombospondin 5) with a role in bridging ECM structures and fibrosis.[31] COMP is one of the most significant DE genes with log2 fold change of +1.7 in DN vs. control fibroblasts but no significant difference between null and control fibroblasts. Here, TNXB is linked to Ehlers-Danlos syndrome.