Decreased N-propeptide cleavage of the type I procollagen N-propeptide can also be caused by defects in the type I procollagen N-proteinase, ADAMTS2, due to biallelic loss-of-function variants in the ADAMTS2 gene, which gives rise to the dermatosparaxis EDS type (dEDS). Here, COL1A2 is linked to Ehlers-Danlos syndrome, dermatosparaxis type.