Ehlers-Danlos Syndrome (EDS) is a heterogeneous group of congenital connective tissue disorders thought to be caused by mutations in genes coding for collagen proteins (i.e. COL3A, COL5A) associated with collagen processing (i.e. PLOD1, ADAMTS2), or for extracellular matrix components (i.e. TNXB)1, 2, 3. The gene discussed is TNXB; the disease is Ehlers-Danlos syndrome.