Glucosylgalactosyl hydroxylysine
Glucosylgalactosyl 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 intermolecliar collagen crosslinks. (OMIM 153454 ). Hydroxylysine-deficient skin collagen is manifested in Ehlers-Danlos syndrome, type VIA, A heritable disorder of connective tissue (PMID 5016372 ).
Structure for HMDB00585 (Glucosylgalactosyl hydroxylysine)
C18H34N2O13
486.4682
486.206089184
(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]oxy}oxan-2-yl]oxy}hexanoic acid
(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]oxy}oxan-2-yl]oxy}hexanoic acid
32448-35-4
UTIRJVJBKWSIOX-SRMFCGEKSA-N
This compound belongs to the class of chemical entities known as fatty acyl glycosides of mono- and disaccharides. These are compounds composed of a mono- or disaccharide moiety linked to one hydroxyl group of a fatty alcohol or of a phosphorylated alcohol (phosphoprenols), a hydroxy fatty acid or to one carboxyl group of a fatty acid (ester linkage) or to an amino alcohol.
Chemical entities
Organic compounds
Lipids and lipid-like moleclies
Fatty Acyls
Fatty acyl glycosides of mono- and disaccharides
Aliphatic heteromonocyclic compounds
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Detected and Quantified
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Solid
Predicted LC-MS/MS Spectrum – 10V, PositiveNot Available
Predicted LC-MS/MS Spectrum – 20V, PositiveNot Available
Predicted LC-MS/MS Spectrum – 40V, PositiveNot Available
Predicted LC-MS/MS Spectrum – 10V, NegativeNot Available
Predicted LC-MS/MS Spectrum – 20V, NegativeNot Available
Predicted LC-MS/MS Spectrum – 40V, NegativeNot Available
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Normal
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None
None
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FDB022130
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109059
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HMDB00585
HMDB00585
5567
122304
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Product: 7,4-Di-O-methylapigenin
- Savolainen ER, Kero M, Pihlajaniemi T, Kivirikko KI: Deficiency of galactosylhydroxylysyl glucosyltransferase, an enzyme of collagen synthesis, in a family with dominant epidermolysis bullosa simplex. N Engl J Med. 1981 Jan 22;304(4):197-204. [PubMed:6449670 ]
- Kelleher PC: Urinary excretion of hydroxyproline, hydroxylysine and hydroxylysine glycosides by patients with Pagets disease of bone and carcinoma with metastases in bone. Clin Chim Acta. 1979 Mar 15;92(3):373-9. [PubMed:436278 ]
- Schroder CH, Monnens LA, van Lith-Zanders HM, Trijbels JM, Veerkamp JH: The urinary excretion of total hydroxylysine and its glycosides in normal persons, and in patients suffering from Alports syndrome–contribution of the peptide-bound fraction. Nephron. 1987;47(4):253-7. [PubMed:3696326 ]
- Schroder CH, Langeveld JP, van Raay-Selten BH, Trijbels FJ, de Graaf R, Veerkamp JH, Monnens LA: Urinary excretion of hydroxylysine and its glycosides in normal persons of different ages–influence of maturation. Int J Pediatr Nephrol. 1985 Oct-Dec;6(4):239-44. [PubMed:2419268 ]
- Schroder CH, Monnens LA, van Lith-Zanders HM, Trijbels JM, Veerkamp JH, Langeveld JP: Urinary excretion of hydroxylysine and its glycosides in Alports syndrome and several other glomerulopathies. Nephron. 1986;44(2):103-7. [PubMed:3774073 ]
- Grazioli V, Alfano M, Stenico A, Casari E: Urinary output of hydroxylysine glycosides and pyridinium cross-links in detecting rat bone collagen turnover rate. FEBS Lett. 1996 Jun 17;388(2-3):134-8. [PubMed:8690072 ]
- Ono S, Shimizu N, Imai T, Rodriguez GP: Urinary collagen metabolite excretion in amyotrophic lateral sclerosis. Muscle Nerve. 2001 Jun;24(6):821-5. [PubMed:11360267 ]
- Rodriguez GP, Claus-Walker J: Measurement of hydroxylysine glycosides in urine and its application to spinal cord injury. J Chromatogr. 1984 Jun 8;308:65-73. [PubMed:6746836 ]
- Szulc P, Seeman E, Delmas PD: Biochemical measurements of bone turnover in children and adolescents. Osteoporos Int. 2000;11(4):281-94. [PubMed:10928217 ]
- Pinnell SR, Krane SM, Kenzora JE, Glimcher MJ: A heritable disorder of connective tissue. Hydroxylysine-deficient collagen disease. N Engl J Med. 1972 May 11;286(19):1013-20. [PubMed:5016372 ]
- (). Bank, Ruud A., Beekman, B., Tenni, R., TeKoppele, Johan M. Pre-column derivatization method for the measurement of glycosylated hydroxylysines of collagenous proteins. Journal of Chromatography, B: Biomedical Sciences and Applications (1997), 703(1 + 2), 267-272. .
- (). Kakimoto, Y., Akazawa, S. Isolation and identification of NG,NG- and NG,N'G-dimethylarginine, N -mono-, di-, and trimethyllysine, and glucosylgalactosyl- and galactosyl- -hydroxylysine from human urine. Journal of Biological Chemistry (1970), 245(21), 5751-8. .