H. Weiler

发表

The severe spontaneous bleeding phenotype in a novel hemophilia A rat model is rescued by platelet FVIII expression.

R. Montgomery, S. Fahs, Q. Shi, 2020, Blood advances.

Knockout Rats via Embryo Microinjection of Zinc-Finger Nucleases

Ignacio Anegon, Bryan Zeitler, Fyodor D Urnov, 2009, Science.

A rat model of severe VWD by elimination of the VWF gene using CRISPR/Cas9

R. Montgomery, S. Fahs, Q. Shi, 2019, Research and practice in thrombosis and haemostasis.

Regulation of inflammation by the protein C system

H. Weiler, 2010, Critical care medicine.

Scientific section designation: THROMBOSIS AND HEMOSTASIS Title: A conditional knockout mouse model reveals endothelial cells as the predominant and possibly exclusive source of plasma factor VIII Short title: FVIII is synthesized by endothelial cells

R. Montgomery, S. Fahs, Q. Shi, 2017 .

A conditional knockout mouse model reveals endothelial cells as the principal and possibly exclusive source of plasma factor VIII.

R. Montgomery, S. Fahs, Q. Shi, 2014, Blood.

Advances in Clinical and Basic Science of Coagulation: Illustrated abstracts of the 9th Chapel Hill Symposium on Hemostasis

C. Maas, W. Lam, A. Sood, 2018, Research and practice in thrombosis and haemostasis.

Calsarcin-2 deficiency increases exercise capacity in mice through calcineurin/NFAT activation.

E. Olson, R. Bassel-Duby, H. Katus, 2008, The Journal of clinical investigation.

Myospryn Is a Direct Transcriptional Target for MEF2A That Encodes a Striated Muscle, α-Actinin-interacting, Costamere-localized Protein*

J. Richardson, J. Durham, F. Naya, 2006, Journal of Biological Chemistry.

Myospryn is a calcineurin‐interacting protein that negatively modulates slow‐fiber‐type transformation and skeletal muscle regeneration

S. Kandarian, F. Naya, Min Y. Cho, 2011, FASEB journal : official publication of the Federation of American Societies for Experimental Biology.

Variable phenotypic penetrance of thrombosis in adult mice after tissue-selective and temporally controlled Thbd gene inactivation.

H. Weiler, T. V. van Mens, J. Foeckler, 2017, Blood advances.

Hyperantithrombotic, noncytoprotective Glu149Ala-activated protein C mutant.

José A Fernández, J. Griffin, Xia V. Yang, 2009, Blood.

The basic helix-loop-helix transcription factor capsulin controls spleen organogenesis.

E. Olson, J. Richardson, Jian-rong Lu, 2000, Proceedings of the National Academy of Sciences of the United States of America.

Mouse models of thrombosis: thrombomodulin

H. Weiler, 2004, Thrombosis and Haemostasis.

Thrombomodulin

B. Isermann, H. Weiler, B. Isermann, 2003, Journal of thrombosis and haemostasis : JTH.

Factor V Leiden is associated with increased sperm count

U. N. Joensen, N. Jørgensen, E. Rajpert-De Meyts, 2017, Human reproduction.

Tissue factor pathway inhibitor is required for cerebrovascular development in mice.

R. Westrick, D. Ginsburg, H. Weiler, 2020, Blood.

Targeting factor VIII expression to platelets for hemophilia A gene therapy does not induce an apparent thrombotic risk in mice

R. Montgomery, Q. Shi, J. G. Mattson, 2017, Journal of thrombosis and haemostasis : JTH.

Myeloid cell–synthesized coagulation factor X dampens antitumor immunity

P. Wilgenbus, W. Ruf, H. Weiler, 2019, Science Immunology.

Role of thrombomodulin expression on hematopoietic stem cells

H. Weiler, J. May, M. Zogg, 2020, Journal of thrombosis and haemostasis : JTH.

Cause-effect relation between hyperfibrinogenemia and vascular disease.

S. Lord, B. Kerlin, B. Isermann, 2004, Blood.

Thrombosis risk modification in transgenic mice containing the human fibrinogen thrombin‐binding γ′ chain sequence

H. Weiler, M. Mosesson, B. Cooley, 2009, Journal of thrombosis and haemostasis : JTH.

Thrombin promotes diet-induced obesity through fibrin-driven inflammation

J. Luyendyk, N. Mackman, S. Divanovic, 2017, The Journal of clinical investigation.

A thrombin-PAR1/2 feedback loop amplifies thromboinflammatory endothelial responses to the viral RNA analogue poly(I:C).

N. Mackman, S. Subramaniam, H. Weiler, 2021, Blood advances.

Thrombomodulin is a determinant of metastasis through a mechanism linked to the thrombin binding domain but not the lectin-like domain.

B. Monia, C. Spek, K. Queiroz, 2011, Blood.

Endogenous EPCR/aPC-PAR1 signaling prevents inflammation-induced vascular leakage and lethality.

José A Fernández, J. Griffin, F. Castellino, 2009, Blood.

Embryogenesis and gene targeting of coagulation factors in mice.

H. Weiler, R. Sood, 2003, Best practice & research. Clinical haematology.

Myozap Deficiency Promotes Adverse Cardiac Remodeling via Differential Regulation of Mitogen-activated Protein Kinase/Serum-response Factor and β-Catenin/GSK-3β Protein Signaling*

R. Lüllmann-Rauch, N. Frey, H. Weiler, 2015, The Journal of Biological Chemistry.

Characterization of a Mouse Model for Thrombomodulin Deficiency

Jeffrey I. Weitz, Mark J. Post, Michael W. Mosesson, 2001, Arteriosclerosis, thrombosis, and vascular biology.

Defective collagen binding and increased bleeding in a murine model of von Willebrand disease affecting collagen IV binding

H. Weiler, J. Foeckler, S. Kalloway, 2019, Journal of thrombosis and haemostasis : JTH.

Contributions of thrombin targets to tissue factor‐dependent metastasis in hyperthrombotic mice

A. Gruber, N. Mackman, F. Castellino, 2014, Journal of thrombosis and haemostasis : JTH.

A Thrombomodulin Mutation that Impairs Active Protein C Generation Is Detrimental in Severe Pneumonia-Derived Gram-Negative Sepsis (Melioidosis)

T. van der Poll, W. Wiersinga, C. van ′t Veer, 2014, PLoS neglected tropical diseases.

disease Cause-effect relation between hyperfibrinogenemia and vascular

S. Lord, B. Kerlin, B. Isermann, 2013 .

Thrombomodulin is required for the antithrombotic activity of thrombin mutant W215A/E217A in a mouse model of arterial thrombosis.

E. Di Cera, H. Weiler, D. Tollefsen, 2012, Thrombosis research.

Combined tissue factor pathway inhibitor and thrombomodulin deficiency produces an augmented hypercoagulable state with tissue‐specific fibrin deposition

H. Weiler, A. Mast, B. Cooley, 2007, Journal of thrombosis and haemostasis : JTH.

Factor V Leiden polymorphism modifies sepsis outcome: Evidence from animal studies

B. Kerlin, H. Weiler, Mary C. Lytle, 2004, Critical care medicine.

Coagulation and Fibrinolysis in Genetically Modified Mice

B. Isermann, H. Weiler, 2004 .

Cause-effect relation between hyperfibrinogenemia and vascular disease.

S. Lord, B. Kerlin, B. Isermann, 2004, Blood.

The Lectin-like Domain of Thrombomodulin Confers Protection from Neutrophil-mediated Tissue Damage by Suppressing Adhesion Molecule Expression via Nuclear Factor κB and Mitogen-activated Protein Kinase Pathways

P. Hellings, S. Pollefeyt, D. Collen, 2002, The Journal of experimental medicine.

Tissue-restricted expression of thrombomodulin in the placenta rescues thrombomodulin-deficient mice from early lethality and reveals a secondary developmental block.

B. Isermann, H. Weiler, Sara B. Hendrickson, 2001, Development.

Quantitative Changes in Gimap3 and Gimap5 Expression Modify Mitochondrial DNA Segregation in Mice

A. Kastaniotis, J. Hiltunen, T. Nyman, 2015, Genetics.

Inflammation-associated activation of coagulation and immune regulation by the protein C pathway.

H. Weiler, 2014, Thrombosis research.

Synthesis and initial pharmacology of dual-targeting ligands for putative complexes of integrin αVβ3 and PAR2.

Jieqing Zhu, H. Weiler, T. Holyst, 2020, RSC medicinal chemistry.

Synthesis and Initial Pharmacology of Heterobivalent Ligands Targeting Putative Complexes of Integrin αVβ3 and PAR2

Jieqing Zhu, S. Subramaniam, H. Weiler, 2019 .

Factor V Leiden mutation enhances fibrin formation and dissolution in vivo in a human endotoxemia model.

M. Borggrefe, B. Jilma, H. Weiler, 2010, Blood.

Thrombomodulin

B. Isermann, H. Weiler, 2003, Reactions Weekly.

Structural and Functional Studies to Define the Molecular Basis by Which Platelet Factor 4 (PF4) Increases Survival of Mice in Lipopolysaccharide (LPS)-Induced Endotoxicity

M. Poncz, H. Weiler, M. Kowalska, 2008 .

Coagulation factor V mediates inhibition of tissue factor signaling by activated protein C in mice.

José A Fernández, J. Griffin, M. Hessner, 2015, Blood.

Comparison of platelet‐derived and plasma factor VIII efficacy using a novel native whole blood thrombin generation assay

R. Montgomery, Q. Shi, H. Weiler, 2015, Journal of thrombosis and haemostasis : JTH.

Thromboelastometry assessment of hemostatic properties in various murine models with coagulopathy and the effect of factor VIII therapeutics

R. Montgomery, Juan Fang, Q. Shi, 2021, Journal of thrombosis and haemostasis : JTH.

Fetal gene defects precipitate platelet-mediated pregnancy failure in factor V Leiden mothers

R. Westrick, S. Coughlin, E. Kerschen, 2007, The Journal of experimental medicine.

1A.1 Animal model of fetal loss in factor V Leiden carriers: pathogenic mechanism and therapeutic targets

H. Weiler, R. Sood, 2007 .

Macrophage protease-activated receptor 2 regulates fetal liver erythropoiesis in mice.

W. Ruf, H. Weiler, S. Reyda, 2018, Blood advances.

Coagulation Factor IIIa (f3a) Knockdown in Zebrafish Leads to Defective Angiogenesis and Mild Bleeding Phenotype

S. Subramaniam, H. Weiler, R. Ramchandran, 2021, bioRxiv.

Activated protein C in sepsis: the promise of nonanticoagulant activated protein C

W. Ruf, H. Weiler, 2008, Current opinion in hematology.

Role of fibrinogen‐ and platelet‐mediated hemostasis in mouse embryogenesis and reproduction

B. Isermann, H. Weiler, J. Degen, 2004, Journal of thrombosis and haemostasis : JTH.

allelescauses noncell-specific deletion of floxed Germ line activation of the Tie2 and SMMHC

E. Radaelli, J. Haigh, C. Sigmund, 2012 .

Modulation of sepsis outcome with variants of activated protein C

E. Kerschen, H. Weiler, 2009, Journal of thrombosis and haemostasis : JTH.

Survival advantage of heterozygous factor V Leiden carriers in murine sepsis

E. Kerschen, H. Weiler, M. Zogg, 2015, Journal of thrombosis and haemostasis : JTH.

Bleeding diathesis in mice lacking JAK2 in platelets.

M. L. Schulte, Nathan Eaton, S. Subramaniam, 2020, Blood advances.

High-resolution mapping of the polyclonal immune response to the human platelet alloantigen HPA-1a (PlA1).

P. Newman, Jieqing Zhu, H. Weiler, 2018, Blood advances.

High-resolution mapping of the polyclonal immune response to the human platelet alloantigen HPA-1a (Pl A1 )

P. Newman, Jieqing Zhu, H. Weiler, 2018 .

Protease‐ and cell type–specific activation of protease‐activated receptor 2 in cutaneous inflammation

Sandra Ritz, M. Bros, W. Ruf, 2022, Journal of thrombosis and haemostasis : JTH.

EPCR-dependent PAR2 activation by the blood coagulation initiation complex regulates LPS-triggered interferon responses in mice.

J. Griffin, M. Hessner, E. Kerschen, 2015, Blood.

THROMBOSIS AND HEMOSTASIS EPCR-dependent PAR 2 activation by the blood coagulation initiation complex regulates LPS-triggered interferon responses in mice

J. Griffin, M. Hessner, E. Kerschen, 2015 .

Blocking hemophilic arthropathy.

Q. Shi, H. Weiler, 2022, Blood.

Intraperitoneal administration of activated protein C prevents postsurgical adhesion band formation.

P. Dinarvand, S. M. Hassanian, H. Weiler, 2013, Blood.

The thrombomodulin–protein C system is essential for the maintenance of pregnancy

B. Isermann, N. Mackman, R. Pawlinski, 2003, Nature Medicine.

hemostasis to hemophilia A mice with preexisting FVIII immunity genetically modified to express factor VIII in platelets restores Syngeneic transplantation of hematopoietic stem cells that are

P. Morateck, R. Montgomery, S. Fahs, 2013 .

Syngeneic transplantation of hematopoietic stem cells that are genetically modified to express factor VIII in platelets restores hemostasis to hemophilia A mice with preexisting FVIII immunity.

P. Morateck, R. Montgomery, S. Fahs, 2008, Blood.

Factor VIII ectopically targeted to platelets is therapeutic in hemophilia A with high-titer inhibitory antibodies.

J. Gorski, P. Morateck, R. Montgomery, 2006, The Journal of clinical investigation.

Nongenotoxic antibody-drug conjugate conditioning enables safe and effective platelet gene therapy of hemophilia A mice.

S. Subramaniam, Q. Shi, H. Weiler, 2019, Blood advances.

TGF-β1 along with other platelet contents augments Treg cells to suppress anti-FVIII immune responses in hemophilia A mice.

M. Hessner, R. Aster, Jianda Hu, 2016, Blood advances.

Targeting FVIII expression to endothelial cells regenerates a releasable pool of FVIII and restores hemostasis in a mouse model of hemophilia A.

R. Montgomery, S. Fahs, Q. Shi, 2010, Blood.

Caveolin-1-dependent apoptosis induced by fibrin degradation products.

T. Kang, B. Isermann, E. Kerschen, 2009, Blood.

Activated protein C protects against diabetic nephropathy by inhibiting endothelial and podocyte apoptosis

C. Esmon, T. Madhusudhan, B. Isermann, 2007, Nature Medicine.

Maternal Par4 and platelets contribute to defective placenta formation in mouse embryos lacking thrombomodulin.

S. Coughlin, B. Isermann, L. Sholl, 2008, Blood.

Expression of human smooth muscle calponin in transgenic mice revealed with a bacterial artificial chromosome.

L. Ashworth, R. Krahe, Jiyuan Chen, 2002, American journal of physiology. Heart and circulatory physiology.

Critical role for Gimap5 in the survival of mouse hematopoietic stem and progenitor cells

X. Dai, H. Weiler, Yuhong Chen, 2011, The Journal of experimental medicine.

Critical role for Gimap 5 in the survival of mouse hematopoietic stem and progenitor cells

X. Dai, H. Weiler, Yuhong Chen, 2011 .

Fetomaternal cross talk in the placental vascular bed: control of coagulation by trophoblast cells.

C. Hillard, H. Weiler, A. Mast, 2006, Blood.

Transmission Ratios at the T-Locus in the Mouse: Inter-and Intra-Male Heterogeneity

H. Weiler, A. Braden, 1964 .

An efficient method to successively introduce transgenes into a given genomic locus in the mouse

S. Fahs, H. Weiler, S. Duncan, 2001, BMC Developmental Biology.

A platelet cloak for tumor cells

H. Weiler, 2005 .

A balance between TFPI and thrombin-mediated platelet activation is required for murine embryonic development.

J. Luyendyk, H. Weiler, A. Mast, 2015, Blood.

Hypercoagulability Inhibits Monocyte Transendothelial Migration Through Protease-Activated Receptor-1–, Phospholipase-Cβ–, Phosphoinositide 3-Kinase–, and Nitric Oxide–Dependent Signaling in Monocytes and Promotes Plaque Stability

D. Frommhold, T. Madhusudhan, B. Isermann, 2009, Circulation.

Genetic and Pharmacological Modifications of Thrombin Formation in Apolipoprotein E-deficient Mice Determine Atherosclerosis Severity and Atherothrombosis Onset in a Neutrophil-Dependent Manner

M. Daemen, C. Esmon, O. Soehnlein, 2013, PloS one.

Endotoxemia and sepsis mortality reduction by non-anticoagulant–activated protein C

Nigel Mackman, Edward J. Kerschen, John H. Griffin, 2007, The Journal of experimental medicine.

Overexpression of activated protein C hampers bacterial dissemination during pneumococcal pneumonia

T. van der Poll, B. Isermann, C. van ′t Veer, 2014, BMC Infectious Diseases.

Activated protein C targets CD8+ dendritic cells to reduce the mortality of endotoxemia in mice.

José A Fernández, J. Griffin, M. Hessner, 2010, The Journal of clinical investigation.

Survival advantage associated with heterozygous factor V Leiden mutation in patients with severe sepsis and in mouse endotoxemia.

J. Dhainaut, B. Kerlin, B. Isermann, 2003, Blood.

Age-dependent vulnerability to endotoxemia is associated with reduction of anticoagulant factors activated protein C and thrombomodulin.

H. Saito, C. Esmon, B. Evers, 2010, Blood.

Nogo-B receptor deficiency causes cerebral vasculature defects during embryonic development in mice.

M. Holtz, Wenquan Hu, H. Weiler, 2016, Developmental biology.

Endothelium-specific loss of murine thrombomodulin disrupts the protein C anticoagulant pathway and causes juvenile-onset thrombosis.

M. Yanagisawa, B. Isermann, Y. Kisanuki, 2001, The Journal of clinical investigation.

mouse embryos lacking thrombomodulin Maternal Par4 and platelets contribute to defective placenta formation in

S. Coughlin, B. Isermann, L. Sholl, 2013 .

Maternal Par 4 and platelets contribute to defective placenta formation in mouse embryos lacking thrombomodulin Running title : Fetal loss of thrombomodulin null mice

S. Coughlin, B. Isermann, L. Sholl, 2008 .

The impact of aberrant von Willebrand factor-GPIbα interaction on megakaryopoiesis and platelets in humanized type 2B von Willebrand disease model mouse

T. Kanaji, R. Montgomery, Y. Morodomi, 2022, Haematologica.

The Adult Murine Intestine is Dependent on Constitutive Laminin-γ1 Synthesis

H. Weiler, M. Battle, J. May, 2019, Scientific Reports.

Thrombomodulin regulates keratinocyte differentiation and promotes wound healing.

Yu-Ting Wu, Hua-Lin Wu, H. Weiler, 2013, The Journal of investigative dermatology.

High-resolutionmapping of the polyclonal immune response to the human platelet alloantigen HPA-1a (Pl)

P. Newman, Jieqing Zhu, H. Weiler, 2018 .

Nogo‐B receptor deficiency increases liver X receptor alpha nuclear translocation and hepatic lipogenesis through an adenosine monophosphate–activated protein kinase alpha–dependent pathway

Jihong Han, Yuanli Chen, Yajun Duan, 2016, Hepatology.

Restricted inactivation of serum response factor to the cardiovascular system.

D. Ginty, K. L. de Mesy Bentley, J. Miano, 2004, Proceedings of the National Academy of Sciences of the United States of America.

High endogenous activated protein C levels attenuates bleomycin‐induced pulmonary fibrosis

T. van der Poll, B. Isermann, K. Borensztajn, 2016, Journal of cellular and molecular medicine.

Thrombomodulin is essential for maintaining quiescence in vascular endothelial cells

I. Biswas, H. Weiler, A. Rezaie, 2021, Proceedings of the National Academy of Sciences.

Fibrinogen alpha and gamma genes and factor VLeiden in children with thromboembolism: results from 2 family-based association studies.

M. Stoll, H. Weiler, U. Nowak‐Göttl, 2008, Blood.

Fibrinogen and genes and factor VLeiden in children with thromboembolism : results from 2 family-based association studies

M. Stoll, H. Weiler, U. Nowak‐Göttl, 2009 .

Pharmacological targeting of the thrombomodulin–protein C pathway mitigates radiation toxicity

José A Fernández, A. Schambach, L. Fink, 2012, Nature Medicine.

Pharmacological targeting of the thrombomodulin – activated protein C pathway mitigates radiation toxicity

José A Fernández, A. Schambach, L. Fink, 2022 .

Multiple receptor-mediated functions of activated protein C

H. Weiler, Hartmut Weiler, 2011, Hämostaseologie.

Endothelial protein C receptor: location…with a pool!

H. Weiler, Hartmut Weiler, 2007, Journal of thrombosis and haemostasis : JTH.

Sucrose non-fermenting related kinase enzyme is essential for cardiac metabolism

S. Palecek, L. Zhong, H. Weiler, 2014, Biology Open.

Thrombomodulin Contributes to Gamma Tocotrienol-Mediated Lethality Protection and Hematopoietic Cell Recovery in Irradiated Mice

M. Boerma, Daohong Zhou, M. Hauer-Jensen, 2015, PloS one.

Tracing the molecular pathogenesis of antiphospholipid syndrome.

H. Weiler, 2008, The Journal of clinical investigation.

Sex-dependent effects of genetic upregulation of activated protein C on delayed effects of acute radiation exposure in the mouse heart, small intestine, and skin

José A Fernández, M. Boerma, Preeti Singh, 2021, PloS one.

Mechanisms for Mortality Reduction by Activated Protein C in Severe Sepsis.

José A Fernández, J. Griffin, S. Coughlin, 2006 .

Impaired survival of peripheral T cells, disrupted NK/NKT cell development, and liver failure in mice lacking Gimap5.

Å. Lernmark, A. Kwitek, M. Hessner, 2008, Blood.

Protective Effect of Activated Protein C in Murine Endotoxemia: Mechanism of Action.

J. Griffin, F. Castellino, E. Kerschen, 2005 .

Thrombomodulin.

B. Isermann, H. Weiler, 2003, Journal of Thrombosis and Haemostasis.

Cause-effect relation between hyperfibrinogenemia and vascular disease

S. Lord, B. Kerlin, B. Isermann, 2004 .

PAR4 antagonists in inflammation

W. Ruf, H. Weiler, 2007 .

The parmodulin NRD-21 is an allosteric inhibitor of PAR1 Gq signaling with improved anti-inflammatory activity and stability.

Kaitlin Kentala, S. Subramaniam, H. Weiler, 2019, Bioorganic & medicinal chemistry.

Activated protein C targets CD8+ dendritic cells to reduce the mortality of endotoxemia in mice.

José A Fernández, J. Griffin, M. Hessner, 2010, The Journal of clinical investigation.

Regulation of inflammation by the protein C system

H. Weiler, 2010, Critical care medicine.

Thrombomodulin is a determinant of metastasis through a mechanism linked to the thrombin binding domain but not the lectin-like domain.

H. Weiler, E. Mullins, J. Degen, 2011, Blood.

Endothelial and Platelet FVIII/VWF Expression - Divergence in Clinical Effect in Murine Models of Hemophilia A with and without FVIII Inhibitory Antibodies.

R. Montgomery, S. Fahs, Q. Shi, 2006 .