Multifunctional enzyme that catalyzes a series of essential post-translational modifications on Lys residues in procollagen . Plays a redundant role in catalyzing the formation of hydroxylysine residues in -Xaa-Lys-Gly- sequences in collagens . Plays a redundant role in catalyzing the transfer of galactose onto hydroxylysine groups, giving rise to galactosyl 5-hydroxylysine . Has an essential role by catalyzing the subsequent transfer of glucose moieties, giving rise to 1,2-glucosylgalactosyl-5-hydroxylysine residues . Catalyzes hydroxylation and glycosylation of Lys residues in the MBL1 collagen-like domain, giving rise to hydroxylysine and 1,2-glucosylgalactosyl-5-hydroxylysine residues . Essential for normal biosynthesis and secretion of type IV collagens (Probable). Essential for normal formation of basement membranes (By similarity).
Rough endoplasmic reticulum . Endoplasmic reticulum lumen . Endoplasmic reticulum membrane ; Peripheral membrane protein ; Lumenal side . Secreted . Secreted, extracellular space . The majority of the secreted protein is associated with the extracellular matrix. .
Ubiquitous (PubMed:9724729). Detected in heart, placenta and pancreas and at lower levels in lung, liver and skeletal muscle (PubMed:9582318, PubMed:9724729).
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