dpy-18 encodes a catalytic alpha subunit of collagen prolyl 4-hydroxylase (P4H) orthologous to human P4H alpha (I) isoform (OMIM: 176710), an alpha subunit of prolyl-4-hydroxylase which is a procollagen modifying enzyme required for exoskeleton formation, morphogenesis and maintenance of body shape; dpy-18 has a critical role in hydroxylation of cuticle collagens during collagen synthesis; dpy-18, in complex with phy-2, is essential for the survival of the C. elegans; dpy-18 mutations alone result in a mild dumpy phenotype; expressed dpy-18 and ten-1 act in parallel influencing the connection between epidermis and muscle during embryonic development; deletion of dpy-18 causes aggregation of collagen IV in body wall muscles in elongated embryos and triggers the loss of tissue integrity in ten-1 mutants; dpy-18 is expressed throughout the hypodermis, head and posterior neurons.
Enables procollagen-proline 4-dioxygenase activity. Involved in several processes, including cuticle development involved in collagen and cuticulin-based cuticle molting cycle; embryo development; and embryonic body morphogenesis. Located in endoplasmic reticulum. Expressed in hypodermis; muscle cell; neurons; and pharynx. Human ortholog(s) of this gene implicated in myopia. Is an ortholog of human P4HA1 (prolyl 4-hydroxylase subunit alpha 1) and P4HA2 (prolyl 4-hydroxylase subunit alpha 2).