Immunogen sequence: SDRPRGRDPV NPEKLLVITV ATAETEGYLR FLRSAEFFNY TVRTLGLGEE WRGGDVARTV GGGQKVRWLK KEMEKYADRE DMIIMFVDSY DVILAGSPTE LLKKFVQSGS RLLFSAESFC WPEWGLAEQY PEVGTGKRFL NSGGFIGFAT TIHQIVRQWK YKDDDDDQLF YTRLYLDPGL REKLSLNLDH KSRIFQNLNG ALDEVVLKFD RNRVRIRNVA YDTLPIVVHG NGPTKLQLNY LGNYVPNGWT PEGGCG; Positive Samples: MCF-7, H460, 22RV1, HepG2, Mouse lung, Rat liver, Rat kidney; Cellular Location: Lumenal side, Peripheral membrane protein, Rough endoplasmic reticulum membrane
The protein encoded by this gene is a membrane-bound homodimeric enzyme that is localized to the cisternae of the rough endoplasmic reticulum. The enzyme (cofactors iron and ascorbate) catalyzes the hydroxylation of lysyl residues in collagen-like peptides. The resultant hydroxylysyl groups are attachment sites for carbohydrates in collagen and thus are critical for the stability of intermolecular crosslinks. Some patients with Ehlers-Danlos syndrome type VIB have deficiencies in lysyl hydroxylase activity.
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Protein Aliases: bone protein I (BP-I); Galactosylhydroxylysine-glucosyltransferase; LH3; lysine hydroxylase 3; lysyl hydroxlase 3; lysyl hydroxylase 2; Lysyl hydroxylase 3; Multifunctional procollagen lysine hydroxylase and glycosyltransferase LH3; Procollagen galactosyltransferase; Procollagen glucosyltransferase; Procollagen glycosyltransferase; procollagen-lysine, 2-oxoglutarate 5-dioxygenase 3; Procollagen-lysine,2-oxoglutarate 5-dioxygenase 3
Gene Aliases: AI414586; LH3; PLOD3