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Literature summary extracted from

  • Wang, C.; Ristiluoma, M.M.; Salo, A.M.; Eskelinen, S.; Myllylae, R.
    Lysyl hydroxylase 3 is secreted from cells by two pathways (2012), J. Cell. Physiol., 227, 668-675.
    View publication on PubMed

Cloned(Commentary)

EC Number Cloned (Comment) Organism
1.14.11.4 gene PLOD3, expression in COS-7 and HT-1080 cells, the enzyme is secreted into the culture medium, expression of enzyme mutants in COS-7 cells Homo sapiens
2.4.1.66 expressed in MDCK, COS-7 and HT-1080 cells Homo sapiens

Protein Variants

EC Number Protein Variants Comment Organism
1.14.11.4 N223S site-directed mutagenesis, the mutant shows 50% reduced lysylhydroxylase activity, while the glycosyltransferase activity is almost abolished Homo sapiens
2.4.1.66 D187A/D189A/D190A/D191A the mutant shows unreduced lysyl hydroxylase 3 activity Homo sapiens
2.4.1.66 D669A the mutant shows no lysyl hydroxylase 3 activity Homo sapiens
2.4.1.66 N23S lysyl hydroxylase 3 activity is reduced by nearly 50% Homo sapiens
2.4.1.66 R729A the mutant shows no lysyl hydroxylase 3 activity Homo sapiens

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
1.14.11.4 cell surface LH3 found on the cell surface bypasses the Golgi complex Homo sapiens 9986
-
1.14.11.4 endoplasmic reticulum associated with membranes in the lumen Homo sapiens 5783
-
1.14.11.4 extracellular LH3 is the only secreted LH isoform, LH3 is secreted by two pathways, one through the Golgi complex leading to EndoH resistant LH3, and the other by-passing the Golgi complex and leading to EndoH sensitive LH3 Homo sapiens
-
-
1.14.11.4 extracellular LH3 is the only secreted LH isoform, secretion of LH3 into the cell medium occurs through both the apical and basolateral plasma membranes of epithelial MDCK cells Canis lupus familiaris
-
-
1.14.11.4 additional information LH3 secretory pathways leading to both surfaces of the cells and the non-polarized secretion of LH3 in MDCK cells Canis lupus familiaris
-
-
1.14.11.4 additional information while the lysyl hydroxylase active site mediates retention of LH3 in the endoplasmic reticulum, the glucosyltransferase active site is required for the secretion of LH3 into the extracellular space Homo sapiens
-
-
2.4.1.66 cell surface
-
 Homo sapiens 9986
-
2.4.1.66 endoplasmic reticulum secretion of the enzyme from the endoplasmic reticulum to the extracellular space occurs via two secretory pathways, which generate two glycoforms. Enzyme molecules found in the cell medium are secreted through the Golgi complex, and the secretion is dependent on lysyl hydroxylase 3 glycosyltransferase activity. Enzyme found on the cell surface bypasses the Golgi complex Homo sapiens 5783
-
2.4.1.66 extracellular
-
 Homo sapiens
-
-

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
1.14.11.4 L-lysine-[procollagen] + 2-oxoglutarate + O2 Canis lupus familiaris
-
 (2S,5R)-5-hydroxy-L-lysine-[procollagen] + succinate + CO2
-
 ?
1.14.11.4 L-lysine-[procollagen] + 2-oxoglutarate + O2 Homo sapiens
-
 (2S,5R)-5-hydroxy-L-lysine-[procollagen] + succinate + CO2
-
 ?
1.14.11.4 L-lysine-[procollagen] + 2-oxoglutarate + O2 Canis lupus familiaris Madin-Darby
-
 (2S,5R)-5-hydroxy-L-lysine-[procollagen] + succinate + CO2
-
 ?

Organism

EC Number Organism UniProt Comment Textmining
1.14.11.4 Canis lupus familiaris
-
-
-
1.14.11.4 Canis lupus familiaris Madin-Darby
-
-
-
1.14.11.4 Homo sapiens O60568 gene Plod3
-
2.4.1.66 Homo sapiens
-
-
-

Source Tissue

EC Number Source Tissue Comment Organism Textmining
1.14.11.4 blood serum
-
 Homo sapiens
-
1.14.11.4 epithelium
-
 Canis lupus familiaris
-
1.14.11.4 kidney
-
 Canis lupus familiaris
-
1.14.11.4 MDCK cell polarized Canis lupus familiaris
-
2.4.1.66 serum
-
 Homo sapiens
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
1.14.11.4 KGIKGIKG + 2-oxoglutarate + O2 a synthetic peptide substrate Canis lupus familiaris ?
-
 ?
1.14.11.4 KGIKGIKG + 2-oxoglutarate + O2 a synthetic peptide substrate Homo sapiens ?
-
 ?
1.14.11.4 KGIKGIKG + 2-oxoglutarate + O2 a synthetic peptide substrate Canis lupus familiaris Madin-Darby ?
-
 ?
1.14.11.4 L-lysine-[procollagen] + 2-oxoglutarate + O2
-
 Canis lupus familiaris (2S,5R)-5-hydroxy-L-lysine-[procollagen] + succinate + CO2
-
 ?
1.14.11.4 L-lysine-[procollagen] + 2-oxoglutarate + O2
-
 Homo sapiens (2S,5R)-5-hydroxy-L-lysine-[procollagen] + succinate + CO2
-
 ?
1.14.11.4 L-lysine-[procollagen] + 2-oxoglutarate + O2
-
 Canis lupus familiaris Madin-Darby (2S,5R)-5-hydroxy-L-lysine-[procollagen] + succinate + CO2
-
 ?
2.4.1.66 additional information lysyl hydroxylase 3 is a post-translational modification enzyme with lysyl hydroxylase collagen galactosyltransferase, and glucosyltransferase activities Homo sapiens ?
-
 ?
2.4.1.66 UDP-glucose + calf skin gelatin
-
 Homo sapiens ?
-
 ?

Synonyms

EC Number Synonyms Comment Organism
1.14.11.4 LH3
-
 Canis lupus familiaris
1.14.11.4 LH3
-
 Homo sapiens
1.14.11.4 lysyl hydroxylase 3
-
 Canis lupus familiaris
1.14.11.4 lysyl hydroxylase 3
-
 Homo sapiens
2.4.1.66 LH3
-
 Homo sapiens
2.4.1.66 lysyl hydroxylase
-
 Homo sapiens

Expression

EC Number Organism Comment Expression
1.14.11.4 Homo sapiens LH activity of LH3 is not reduced by mutations of this DXD motif additional information

General Information

EC Number General Information Comment Organism
1.14.11.4 physiological function lysyl hydroxylase 3 (LH3) is a post-translational modification enzyme with lysyl hydroxylase, collagen galactosyltransferase EC 2.4.1.50, and glucosyltransferase, EC 2.4.1.66, activities, LH3 is able to catalyze the three consecutive reactions required for the formation of unique hydroxylysine-linked carbohydrates, galactosylhydroxylysine and glucosylgalactosyl hydroxylysine, found in collagens and a few other proteins. The active sites for the different activities of the mutifunctional enzyme are localized separately in the C- and the N-terminal parts of the molecule. While the lysyl hydroxylase active site mediates retention of LH3 in the endoplasmic reticulum, the glucosyltransferase active site is required for the secretion of LH3 into the extracellular space Canis lupus familiaris
1.14.11.4 physiological function lysyl hydroxylase 3 (LH3) is a post-translational modification enzyme with lysyl hydroxylase, collagen galactosyltransferase EC 2.4.1.50, and glucosyltransferase, EC 2.4.1.66, activities, LH3 is able to catalyze the three consecutive reactions required for the formation of unique hydroxylysine-linked carbohydrates, galactosylhydroxylysine and glucosylgalactosyl hydroxylysine, found in collagens and a few other proteins. The active sites for the different activities of the mutifunctional enzyme are localized separately in the C- and the N-terminal parts of the molecule. While the lysyl hydroxylase active site mediates retention of LH3 in the endoplasmic reticulum, the glucosyltransferase active site is required for the secretion of LH3 into the extracellular space Homo sapiens