Prolyl 3-hydroxylase 2 is an enzyme that in humans is encoded by the gene P3H2 (formally LEPREL1). This enzyme is involved in the assembly of collagen. Pathogenic mutations in the gene are associated with severe nearsightedness (myopia) and cataracts.
Function As their names suggests, the Prolyl 3-hydroxylase 2 and its relatives Prolyl 3-hydroxylase 1 and 3 function as collagen prolyl 3-hydroxylases. This means they act on the amino acid, proline, located in collagen precursor proteins, and attaches a hydroxyl at the 3-position, converting the proline into 3-hydroxyproline. This seems to function to help the corresponding collagen fibrils bind together and align properly. P3H2 differs from P3H1 in a number of important ways. It is primarily expressed in basement membrane-rich tissues (P3H1 is primarily in fibrillar collagens rich tissues). It is also able to act on type IV collagen (especially COL4A1), unlike P3H1. Both enzymes are able to act on Type I collagen (and COL1A1), but they tend to modify the prolines at different positions. P3H2 also appears to function on its own, while P3H1 forms a complex with two other proteins (CRTAP and CypB).
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