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Leprecan and Prolyl 3-Hydroxylase Activity
Leprecan, a proteoglycan, has demonstrated prolyl hydroxylase activity; prolyl hydroxylases hydroxylate proline residues. Prolyl 3-hydroxylase 1, P3H1, forms a larger complex with CRTAP and cyclophilin B, CyPB, in the endoplasimic reticulum. The complex hydroxylates a single proline residue, Pro986, on collagen chains. Recessive forms of Osteogenesis Imperfecta is partly caused by a mutation in the LEPRE1 gene that encodes prolyl 3-hydroxylase 1; malfunctioning prolyl 3-hydroxylase in leprecan leads to inappropriate collagen folding due to instability caused by the absence of hydroxyproline, the product of hydroxylating a proline residue.