CHAPTER 5: Collagen Hydroxylases

Collagen synthesis requires the modification of procollagen polypeptides by three types of 2-oxoglutarate-dependent dioxygenases: collagen prolyl 4-hydroxylases (C–P4Hs), lysyl hydroxylases (LHs) and prolyl 3-hydroxylases (P3Hs), with each family consisting of three isoenzymes in vertebrates. This review addresses the molecular, functional and structural characteristics of the collagen hydroxylases. Detailed information on the individual in vivo roles of the collagen hydroxylase isoenzymes is also discussed based on the emerging data from mouse models and human diseases caused by mutations in genes coding for collagen hydroxylases.