Toll-Like Receptor 3 Mediates Aortic Stenosis Through a Conserved Mechanism of Calcification.


Journal

Circulation
ISSN: 1524-4539
Titre abrégé: Circulation
Pays: United States
ID NLM: 0147763

Informations de publication

Date de publication:
16 05 2023
Historique:
pmc-release: 16 05 2024
medline: 17 5 2023
pubmed: 5 4 2023
entrez: 4 4 2023
Statut: ppublish

Résumé

Calcific aortic valve disease (CAVD) is characterized by a phenotypic switch of valvular interstitial cells to bone-forming cells. Toll-like receptors (TLRs) are evolutionarily conserved pattern recognition receptors at the interface between innate immunity and tissue repair. Type I interferons (IFNs) are not only crucial for an adequate antiviral response but also implicated in bone formation. We hypothesized that the accumulation of endogenous TLR3 ligands in the valvular leaflets may promote the generation of osteoblast-like cells through enhanced type I IFN signaling. Human valvular interstitial cells isolated from aortic valves were challenged with mechanical strain or synthetic TLR3 agonists and analyzed for bone formation, gene expression profiles, and IFN signaling pathways. Different inhibitors were used to delineate the engaged signaling pathways. Moreover, we screened a variety of potential lipids and proteoglycans known to accumulate in CAVD lesions as potential TLR3 ligands. Ligand-receptor interactions were characterized by in silico modeling and verified through immunoprecipitation experiments. Biglycan ( Here, we identify TLR3 as a central molecular regulator of calcification in valvular interstitial cells and unravel BGN as a new endogenous agonist of TLR3. Posttranslational BGN maturation by xylosyltransferase 1 (XYLT1) is required for TLR3 activation. Moreover, BGN induces the transdifferentiation of valvular interstitial cells into bone-forming osteoblasts through the TLR3-dependent induction of type I IFNs. It is intriguing that This study identifies the BGN-TLR3-IFNAR1 axis as an evolutionarily conserved pathway governing calcification of the aortic valve and reveals a potential therapeutic target to prevent CAVD.

Sections du résumé

BACKGROUND
Calcific aortic valve disease (CAVD) is characterized by a phenotypic switch of valvular interstitial cells to bone-forming cells. Toll-like receptors (TLRs) are evolutionarily conserved pattern recognition receptors at the interface between innate immunity and tissue repair. Type I interferons (IFNs) are not only crucial for an adequate antiviral response but also implicated in bone formation. We hypothesized that the accumulation of endogenous TLR3 ligands in the valvular leaflets may promote the generation of osteoblast-like cells through enhanced type I IFN signaling.
METHODS
Human valvular interstitial cells isolated from aortic valves were challenged with mechanical strain or synthetic TLR3 agonists and analyzed for bone formation, gene expression profiles, and IFN signaling pathways. Different inhibitors were used to delineate the engaged signaling pathways. Moreover, we screened a variety of potential lipids and proteoglycans known to accumulate in CAVD lesions as potential TLR3 ligands. Ligand-receptor interactions were characterized by in silico modeling and verified through immunoprecipitation experiments. Biglycan (
RESULTS
Here, we identify TLR3 as a central molecular regulator of calcification in valvular interstitial cells and unravel BGN as a new endogenous agonist of TLR3. Posttranslational BGN maturation by xylosyltransferase 1 (XYLT1) is required for TLR3 activation. Moreover, BGN induces the transdifferentiation of valvular interstitial cells into bone-forming osteoblasts through the TLR3-dependent induction of type I IFNs. It is intriguing that
CONCLUSIONS
This study identifies the BGN-TLR3-IFNAR1 axis as an evolutionarily conserved pathway governing calcification of the aortic valve and reveals a potential therapeutic target to prevent CAVD.

Identifiants

pubmed: 37013819
doi: 10.1161/CIRCULATIONAHA.122.063481
pmc: PMC10192061
mid: NIHMS1884895
doi:

Substances chimiques

Biglycan 0
Toll-Like Receptor 3 0
TLR3 protein, human 0
TLR3 protein, mouse 0

Types de publication

Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

1518-1533

Subventions

Organisme : NHLBI NIH HHS
ID : R01 HL128550
Pays : United States

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Auteurs

Can Gollmann-Tepeköylü (C)

Department of Cardiac Surgery (C.G.-T., M.G, J.H., S.M., L.P., F.N., E.K., N.F., D. Lobenwein, V.S., J.E., M.G., J.H.), Medical University of Innsbruck, Austria.

Michael Graber (M)

Department of Cardiac Surgery (C.G.-T., M.G, J.H., S.M., L.P., F.N., E.K., N.F., D. Lobenwein, V.S., J.E., M.G., J.H.), Medical University of Innsbruck, Austria.

Jakob Hirsch (J)

Department of Cardiac Surgery (C.G.-T., M.G, J.H., S.M., L.P., F.N., E.K., N.F., D. Lobenwein, V.S., J.E., M.G., J.H.), Medical University of Innsbruck, Austria.

Sophia Mair (S)

Department of Cardiac Surgery (C.G.-T., M.G, J.H., S.M., L.P., F.N., E.K., N.F., D. Lobenwein, V.S., J.E., M.G., J.H.), Medical University of Innsbruck, Austria.

Andreas Naschberger (A)

Institute of Genetic Epidemiology, Department of Genetics and Pharmacology (A.N., F.K., S.C., B.R.), Medical University of Innsbruck, Austria.
Division of Biological and Environmental Sciences and Engineering, King Abdullah University of Science and Technology, Thuwal4 Saudi Arabia (A.N.).

Leo Pölzl (L)

Department of Cardiac Surgery (C.G.-T., M.G, J.H., S.M., L.P., F.N., E.K., N.F., D. Lobenwein, V.S., J.E., M.G., J.H.), Medical University of Innsbruck, Austria.

Felix Nägele (F)

Department of Cardiac Surgery (C.G.-T., M.G, J.H., S.M., L.P., F.N., E.K., N.F., D. Lobenwein, V.S., J.E., M.G., J.H.), Medical University of Innsbruck, Austria.

Elke Kirchmair (E)

Department of Cardiac Surgery (C.G.-T., M.G, J.H., S.M., L.P., F.N., E.K., N.F., D. Lobenwein, V.S., J.E., M.G., J.H.), Medical University of Innsbruck, Austria.
Department of Internal Medicine III (E.D., R.H., A.B., D. Lener, M.S., R.K., G.W., I.T.), Medical University of Innsbruck, Austria.

Gerald Degenhart (G)

Department of Radiology, Core Facility for Micro-CT (G.D., G.F.), Medical University of Innsbruck, Austria.

Egon Demetz (E)

Department of Internal Medicine III (E.D., R.H., A.B., D. Lener, M.S., R.K., G.W., I.T.), Medical University of Innsbruck, Austria.

Richard Hilbe (R)

Department of Internal Medicine III (E.D., R.H., A.B., D. Lener, M.S., R.K., G.W., I.T.), Medical University of Innsbruck, Austria.

Hao-Yu Chen (HY)

Preventive and Genomic Cardiology, McGill University Health Centre Research Institute, Montreal, Quebec, Canada (J.C.E., H.-Y.C., G.T.).

James C Engert (JC)

Preventive and Genomic Cardiology, McGill University Health Centre Research Institute, Montreal, Quebec, Canada (J.C.E., H.-Y.C., G.T.).

Anna Böhm (A)

Department of Internal Medicine III (E.D., R.H., A.B., D. Lener, M.S., R.K., G.W., I.T.), Medical University of Innsbruck, Austria.

Nadja Franz (N)

Department of Cardiac Surgery (C.G.-T., M.G, J.H., S.M., L.P., F.N., E.K., N.F., D. Lobenwein, V.S., J.E., M.G., J.H.), Medical University of Innsbruck, Austria.

Daniela Lobenwein (D)

Department of Cardiac Surgery (C.G.-T., M.G, J.H., S.M., L.P., F.N., E.K., N.F., D. Lobenwein, V.S., J.E., M.G., J.H.), Medical University of Innsbruck, Austria.

Daniela Lener (D)

Department of Internal Medicine III (E.D., R.H., A.B., D. Lener, M.S., R.K., G.W., I.T.), Medical University of Innsbruck, Austria.

Christiane Fuchs (C)

Department Life Science Engineering (C.F., A.W.), University of Applied Sciences Technikum Wien, Vienna, Austria.

Anna Weihs (A)

Department Life Science Engineering (C.F., A.W.), University of Applied Sciences Technikum Wien, Vienna, Austria.

Sonja Töchterle (S)

Institute of Molecular Biology/CMBI (S.T., D.M.), Medical University of Innsbruck, Austria.

Victor Schweiger (V)

Department of Cardiac Surgery (C.G.-T., M.G, J.H., S.M., L.P., F.N., E.K., N.F., D. Lobenwein, V.S., J.E., M.G., J.H.), Medical University of Innsbruck, Austria.

Jonas Eder (J)

Department of Cardiac Surgery (C.G.-T., M.G, J.H., S.M., L.P., F.N., E.K., N.F., D. Lobenwein, V.S., J.E., M.G., J.H.), Medical University of Innsbruck, Austria.

Peter Pietschmann (P)

Division of Cellular and Molecular Pathophysiology, Department of Pathophysiology and Allergy Research, Center for Pathophysiology, Infectiology and Immunology, Medical University of Vienna, Austria (P.P.).

Markus Seifert (M)

Department of Internal Medicine III (E.D., R.H., A.B., D. Lener, M.S., R.K., G.W., I.T.), Medical University of Innsbruck, Austria.

Florian Kronenberg (F)

Institute of Genetic Epidemiology, Department of Genetics and Pharmacology (A.N., F.K., S.C., B.R.), Medical University of Innsbruck, Austria.

Stefan Coassin (S)

Institute of Genetic Epidemiology, Department of Genetics and Pharmacology (A.N., F.K., S.C., B.R.), Medical University of Innsbruck, Austria.

Michael Blumer (M)

Institute of Clinical and Functional Anatomy, Innsbruck Medical University, Austria (M.B.).

Hubert Hackl (H)

Institute of Bioinformatics (H.H.), Medical University of Innsbruck, Austria.

Dirk Meyer (D)

Institute of Molecular Biology/CMBI (S.T., D.M.), Medical University of Innsbruck, Austria.

Gudrun Feuchtner (G)

Department of Radiology, Core Facility for Micro-CT (G.D., G.F.), Medical University of Innsbruck, Austria.

Rudolf Kirchmair (R)

Department of Internal Medicine III (E.D., R.H., A.B., D. Lener, M.S., R.K., G.W., I.T.), Medical University of Innsbruck, Austria.

Jakob Troppmair (J)

Daniel Swarovski Research Laboratory, Department of Visceral, Transplant and Thoracic Surgery (J.T.), Medical University of Innsbruck, Austria.

Markus Krane (M)

Department of Cardiovascular Surgery, German Heart Center Munich at the Technical University Munich, Germany (M.K.).

Günther Weiss (G)

Department of Internal Medicine III (E.D., R.H., A.B., D. Lener, M.S., R.K., G.W., I.T.), Medical University of Innsbruck, Austria.

Sotirios Tsimikas (S)

Division of Cardiovascular Diseases, University of California, San Diego, La Jolla (S.T.).

George Thanassoulis (G)

Preventive and Genomic Cardiology, McGill University Health Centre Research Institute, Montreal, Quebec, Canada (J.C.E., H.-Y.C., G.T.).

Michael Grimm (M)

Department of Cardiac Surgery (C.G.-T., M.G, J.H., S.M., L.P., F.N., E.K., N.F., D. Lobenwein, V.S., J.E., M.G., J.H.), Medical University of Innsbruck, Austria.

Bernhard Rupp (B)

Institute of Genetic Epidemiology, Department of Genetics and Pharmacology (A.N., F.K., S.C., B.R.), Medical University of Innsbruck, Austria.

Lukas A Huber (LA)

Institute of Cell Biology (L.A.H.), Medical University of Innsbruck, Austria.
Austrian Drug Screening Institute, ADSI, Innsbruck (L.A.H.).

Shen-Ying Zhang (SY)

St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, Rockefeller University, New York, NY (S.-Y.Z., J.-L.C.).
Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, Necker Hospital for Sick Children, Paris, France (S.-Y.Z., J.-L.C.).
University of Paris, Imagine Institute, France (S.-Y.Z., J.-L.C.).

Jean-Laurent Casanova (JL)

St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, Rockefeller University, New York, NY (S.-Y.Z., J.-L.C.).
Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, Necker Hospital for Sick Children, Paris, France (S.-Y.Z., J.-L.C.).
University of Paris, Imagine Institute, France (S.-Y.Z., J.-L.C.).
Howard Hughes Medical Institute, New York, NY (J.-L.C.).

Ivan Tancevski (I)

Department of Internal Medicine III (E.D., R.H., A.B., D. Lener, M.S., R.K., G.W., I.T.), Medical University of Innsbruck, Austria.

Johannes Holfeld (J)

Department of Cardiac Surgery (C.G.-T., M.G, J.H., S.M., L.P., F.N., E.K., N.F., D. Lobenwein, V.S., J.E., M.G., J.H.), Medical University of Innsbruck, Austria.

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