ArticleThe Journal of biological chemistry2009
Reduction of lysyl hydroxylase 3 causes deleterious changes in the deposition and organization of extracellular matrix.
Article in The Journal of biological chemistry, 2009. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 35 papers.
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Who cites it
35 citing papers in PubMed, 55 citations in OpenAlex.
- Succinate supplementation ameliorates musculoskeletal defects caused by PLOD3 mutations in a BCARD syndrome model.Genome medicine · 2026Article
- FAM20A Deficiency Drives Transcriptomic Dysregulation and Functional Impairment in Gingival Fibroblasts.Cell proliferation · 2026Article
- Mechanobiological Dynamics-Inspired Mechanomodulatory Biomaterials.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2026Review
- Ocular Tissue-Specific Amino Acid Metabolism in Gyrate Atrophy.Advances in experimental medicine and biology · 2025Review
- Glycosylation Modulates the Structure and Functions of Collagen: A Review.Molecules (Basel, Switzerland) · 2024Review
- Features of Congenital Arthrogryposis Due to Abnormalities in Collagen Homeostasis, a Scoping Review.International journal of molecular sciences · 2023Article
- Comprehensive Analysis of the Expression, Prognosis, and Biological Significance of PLOD Family in Bladder Cancer.International journal of general medicine · 2023Article
- Ivermectin Affects Neutrophil-Induced Inflammation through Inhibition of Hydroxylysine but Stimulation of Cathepsin G and Phenylalanine Secretion.Biomedicines · 2022Article
- Metabolomics analysis of plasma samples of patients with fibromyalgia and electromagnetic sensitivity using GC-MS technique.Scientific reports · 2022Article
- Article
- Perspectives on pediatric congenital aortic valve stenosis: Extracellular matrix proteins, post translational modifications, and proteomic strategies.Frontiers in cardiovascular medicine · 2022Review
- Overexpressing PLOD Family Genes Predict Poor Prognosis in Pancreatic Cancer.International journal of general medicine · 2022Article
- Article
- Dystrophic Epidermolysis Bullosa: Secondary Disease Mechanisms and Disease Modifiers.Frontiers in genetics · 2021Review
- Inhibition of Neutrophil Secretion Upon Adhesion as a Basis for the Anti-Inflammatory Effect of the Tricyclic Antidepressant Imipramine.Frontiers in pharmacology · 2021Article
- Modulation of receptor binding to collagen by glycosylated 5-hydroxylysine: Chemical biology approaches made feasible by Carpino's Fmoc group.Peptide science (Hoboken, N.J.) · 2020Article
- Histological and Gene Expression Analysis of the Effects of Menopause Status and Hormone Therapy on the Vaginal Introitus and Labia Majora.Journal of clinical medicine research · 2019Article
- [Syndromes with skin fragility].Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete · 2019Review
- Overexpression of LH3 reduces the incidence of hypertensive intracerebral hemorrhage in mice.Journal of cerebral blood flow and metabolism : official journal of the International Society of Cerebral Blood Flow and Metabolism · 2019Article
- Molecular architecture of the multifunctional collagen lysyl hydroxylase and glycosyltransferase LH3.Nature communications · 2018Article
Corrections and comments
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Authors and funding
9 authors at 4 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Lysyl hydroxylase 3 (LH3) is a multifunctional enzyme possessing lysyl hydroxylase, collagen galactosyltransferase, and glucosyltransferase (GGT) activities. We report here an important role for LH3 in the organization of the extracellular matrix (ECM) and cytoskeleton. Deposition of ECM was affected in heterozygous LH3 knock-out mouse embryonic fibroblasts (MEF(+/-)) and in skin fibroblasts collected from a member of a Finnish epidermolysis bullosa simplex (EBS) family known to be deficient in GGT activity. We show the GGT deficiency to be due to a transcriptional defect in one LH3 allele. The ECM abnormalities also lead to defects in the arrangement of the cytoskeleton in both cell lines. Ultrastructural abnormalities were observed in the skin of heterozygous LH3 knock-out mice indicating that even a moderate decrease in LH3 has deleterious consequences in vivo. The LH3 null allele in the EBS family member and the resulting abnormalities in the organization of the extracellular matrix, similar to those found in MEF(+/-), may explain the correlation between the severity of the phenotype and the decrease in GGT activity reported in this family.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.