COL1A2 and Ehlers-Danlos syndrome: Human patients with mutations affecting N-terminal processing of type I procollagen suffer from Ehlers-Danlos syndrome (EDS) type VII, a disease characterized by joint hypermobility, skin hyperextensibility, and dislocations (arthrochalasia type, formally type VIIA and VIIB) or skin fragility (type VIIC/dermatosparatic type; Byers et al., 1997; Colige et al., 1999; Giunta et al., 1999; Smith et al., 1992).