Accession NumberHMDB00585
Common_NameGlucosylgalactosyl hydroxylysine
DescriptionGlucosylgalactosyl hydroxylysine is a Glycoside of hydroxylysine. Hydroxylation of lysine is an important post-translational modification of collagen, (PMID 10928217) a reaction catalyzed by the enzyme Lysyl hydroxylase (EC 1.14.11.4) forming hydroxylysine in collagens and other proteins with collagen-like amino acid sequences, by the hydroxylation of lysine residues in X-lys-gly sequences. The hydroxylysine residues formed in the lysyl hydroxylase reaction have 2 important functions: first, their hydroxy groups serve as sites of attachment for carbohydrate units, either the monosaccharide galactose or the disaccharide glucosylgalactose; and second, they are essential for the stability of the intermolecular collagen crosslinks. (OMIM 153454) Hydroxylysine-deficient skin collagen is manifested in Ehlers-Danlos syndrome, type VIA, A heritable disorder of connective tissue (PMID 5016372)
Chemical_IUPAC_Name(2S)-2,6-diamino-5-[(2R,3R,4S,5R,6R)-4,5-dihydroxy-6-(hydroxymethyl)-3-[(2R,3R,5S,6R)-3,4,5-trihydroxy-6-(hydroxymethyl)oxan-2-yl]oxyoxan-2-yl]oxyhexanoic acid
Chemical FormulaC18H34N2O13
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