Multifunctional procollagen lysine hydroxylase and glycosyltransferase LH3 [Includes: Procollagen-lysine,2-oxoglutarate 5-dioxygenase 3

Definition:

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).

References:

[1]. Kati Rautavuoma, et al. Characterization of three fragments that constitute the monomers of the human lysyl hydroxylase isoenzymes 1-3. The 30-kDa N-terminal fragment is not required for lysyl hydroxylase activity. J Biol Chem. 2002 Jun 21;277(25):23084-91. [Content Brief]

[2]. Antti M Salo, et al. A connective tissue disorder caused by mutations of the lysyl hydroxylase 3 gene. Am J Hum Genet. 2008 Oct;83(4):495-503. [Content Brief]

[3]. K Passoja, et al. Cloning and characterization of a third human lysyl hydroxylase isoform. Proc Natl Acad Sci U S A. 1998 Sep 1;95(18):10482-6. [Content Brief]

[4]. Luigi Scietti, et al. Molecular architecture of the multifunctional collagen lysyl hydroxylase and glycosyltransferase LH3. Nat Commun. 2018 Aug 8;9(1):3163. [Content Brief]

[5]. Maija Risteli, et al. Lysyl hydroxylase 3 modifies lysine residues to facilitate oligomerization of mannan-binding lectin. PLoS One. 2014 Nov 24;9(11):e113498. [Content Brief]

[6]. J Heikkinen, et al. Lysyl hydroxylase 3 is a multifunctional protein possessing collagen glucosyltransferase activity. J Biol Chem. 2000 Nov 17;275(46):36158-63. [Content Brief]

[7]. Chunguang Wang, et al. The third activity for lysyl hydroxylase 3: galactosylation of hydroxylysyl residues in collagens in vitro. Matrix Biol. 2002 Nov;21(7):559-66. [Content Brief]

[8]. M Valtavaara, et al. Primary structure, tissue distribution, and chromosomal localization of a novel isoform of lysyl hydroxylase (lysyl hydroxylase 3). J Biol Chem. 1998 May 22;273(21):12881-6. [Content Brief]

[9]. Chunguang Wang, et al. The glycosyltransferase activities of lysyl hydroxylase 3 (LH3) in the extracellular space are important for cell growth and viability. J Cell Mol Med. 2009 Mar;13(3):508-21. [Content Brief]

[10]. Chunguang Wang, et al. Identification of amino acids important for the catalytic activity of the collagen glucosyltransferase associated with the multifunctional lysyl hydroxylase 3 (LH3). J Biol Chem. 2002 May 24;277(21):18568-73. [Content Brief]

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