Glycosylation catalyzed by lysyl hydroxylase 3 is essential for basement membranes

@article{Ruotsalainen2006GlycosylationCB,
  title={Glycosylation catalyzed by lysyl hydroxylase 3 is essential for basement membranes},
  author={Heli Ruotsalainen and Laura Sipil{\"a} and Miia H. Vapola and Raija T. Sormunen and Antti M. Salo and Lahja Uitto and Derry K. Mercer and Simon P. Robins and Maija Risteli and Attila Asz{\'o}di and Reinhard F{\"a}ssler and Raili Myllylä},
  journal={Journal of Cell Science},
  year={2006},
  volume={119},
  pages={625 - 635}
}
Lysyl hydroxylase 3 (LH3) is a multifunctional enzyme possessing lysyl hydroxylase (LH), hydroxylysyl galactosyltransferase (GT) and galactosylhydroxylysyl glucosyltransferase (GGT) activities in vitro. To investigate the in vivo importance of LH3-catalyzed lysine hydroxylation and hydroxylysine-linked glycosylations, three different LH3-manipulated mouse lines were generated. Mice with a mutation that blocked only the LH activity of LH3 developed normally, but showed defects in the structure… 
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Expanding the lysyl hydroxylase toolbox: New insights into the localization and activities of lysyl hydroxylase 3 (LH3)
TLDR
It is shown that growth cone migration depends critically on the LH3 glycosyltransferase domain, but, unlike the other isoforms, LH3 is also found in the extracellular space in some tissues, suggesting that it may have a role in matrix remodeling.
Expression of lysyl hydroxylases and functions of lysyl hydroxylase 3 in mice
TLDR
It is demonstrated that hydroxylysine-linked glycosylations are critical for the secretion of type IV collagen and its assembly into basement membranes, and for the assembly and distribution of type VI collagen.
Reduction of Lysyl Hydroxylase 3 Causes Deleterious Changes in the Deposition and Organization of Extracellular Matrix*
TLDR
An important role for LH3 in the organization of the extracellular matrix (ECM) and cytoskeleton is reported and the GGT deficiency is shown to be due to a transcriptional defect in one LH3 allele.
Expression of lysyl hydroxylases and characterization of a novel disorder caused by mutations in the lysyl hydroxylase 3 gene
TLDR
It was shown that a defect in lysyl hydroxylase 3 catalyzed modifications leads to a novel disorder, which shares features with many other connective tissue disorders.
Lysyl Hydroxylase 3 Modifies Lysine Residues to Facilitate Oligomerization of Mannan-Binding Lectin
TLDR
It is shown for the first time that LH3 is essential for catalyzing formation of the glucosylgalactosylhydroxylysines of mannan-binding lectin (MBL), the first component of the lectin pathway of complement activation.
Lysyl Hydroxylase 3 Glucosylates Galactosylhydroxylysine Residues in Type I Collagen in Osteoblast Culture*
TLDR
The results from this study clearly indicate that the major function of LH3 in osteoblasts is to glucosylate galactosylhydroxylysine residues intype I collagen and that an impairment of this LH3 function significantly affects type I collagen fibrillogenesis.
A connective tissue disorder caused by mutations of the lysyl hydroxylase 3 gene.
Lysyl hydroxylase 3 is secreted from cells by two pathways
TLDR
It is revealed that the secretion of LH3 from the ER to the extracellular space occurs via two secretory pathways, which generate two glycoforms, and the secretion is dependent on LH3 glycosyltransferase activity.
The glycosyltransferase activities of lysyl hydroxylase 3 (LH3) in the extracellular space are important for cell growth and viability
TLDR
The data clearly indicate that the deficiency of LH3 glycosyltransferase activities, especially in the extracellular space, causes growth arrest revealing the importance of the glycosy transferase activities of LH2 for cell growth and viability, and identifying LH3 as a potential target for medical applications, such as cancer therapy.
The Activities of Lysyl Hydroxylase 3 (LH3) Regulate the Amount and Oligomerization Status of Adiponectin
TLDR
It is demonstrated for the first time that LH3 modifies the lysine residues in the collagenous domain of adiponectin, which has important roles in glucose and lipid metabolism and inflammation and thus might be a potential candidate for therapeutic applications in diseases associated with obesity and insulin resistance.
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