Dorina Roem

Iron-Driven Alterations on Red Blood Cell-Derived Microvesicles Amplify Coagulation during Hemolysis via the Intrinsic Tenase Complex

Laura Delvasto-Núñez, Dorina Roem, Kamran Bakhtiari, Gerard van Mierlo et autres

Abstract Hemolytic disorders characterized by complement-mediated intravascular hemolysis, such as autoimmune hemolytic anemia and paroxysmal nocturnal hemoglobinuria, are often complicated by life-threatening thromboembolic complications. Severe hemolytic episodes result in the release of red blood cell (RBC)-derived proinflammatory and oxidatively reactive mediators (e.g., …

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Iron-Driven Alterations on Red Blood Cell-Derived Microvesicles Amplify Coagulation during Hemolysis via the Intrinsic Tenase Complex

Ilse Jongerius, Sacha Zeerleder, Laura Delvasto-Núñez, Dorina Roem et autres

Abstract Hemolytic disorders characterized by complement-mediated intravascular hemolysis, such as autoimmune hemolytic anemia and paroxysmal nocturnal hemoglobinuria, are often complicated by life-threatening thromboembolic complications. Severe hemolytic episodes result in the release of red blood cell (RBC)-derived proinflammatory and oxidatively reactive mediators (e.g., …

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Molecular Basis for Bordetella pertussis Interference with Complement, Coagulation, Fibrinolytic, and Contact Activation Systems: the Cryo-EM Structure of the Vag8-C1 Inhibitor Complex

Arun Dhillon, Justin C. Deme, Emily Furlong, Dorina Roem et autres

The structure of a 10-kDa protein complex is one of the smallest to be determined using cryo-electron microscopy at high resolution. The structure reveals that C1-INH is sequestered in an inactivated state by burial of the reactive center loop in Vag8. By …

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Sugar Matters: Improving In Vivo Clearance Rate of Highly Glycosylated Recombinant Plasma Proteins for Therapeutic Use

Sacha Zeerleder, Ruchira Engel, Tao Zhang, Dorina Roem et autres

Correct glycosylation of proteins is essential for production of therapeutic proteins as glycosylation is important for protein solubility, stability, half-life and immunogenicity. The heavily glycosylated plasma protein C1-inhibitor (C1-INH) is used in treatment of hereditary angioedema attacks. In this study, we used …

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Molecular basis for B. pertussis interference with complement, coagulation, fibrinolytic and contact activation systems: The cryo-EM structure of the Vag8-C1 inhibitor complex

Arun Dhillon, Justin C. Deme, Emily Furlong, Dorina Roem et autres

Abstract Complement, contact activation, coagulation, and fibrinolysis are serum protein cascades that need strict regulation to maintain human health. Serum glycoprotein, C1-inhibitor (C1-INH) is a key regulator (inhibitor) of serine proteases of all the above-mentioned pathways. Recently, an autotransporter protein, Virulence Associated …

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SERPING1 mutation update: Mutation spectrum and C1 Inhibitor phenotypes

D. Ponard, Christine Gaboriaud, Delphine Charignon, Arije Ghannam et autres

C1 inhibitor (C1Inh) deficiency is responsible for hereditary angioedema (C1-INH-HAE) and caused by variants of the SERPING1/C1INH/C1NH gene. C1Inh is the major control of kallikrein-kinin system. C1Inh deficiency leads to its uncontrolled activation, with subsequent generation of the vasoactive peptide bradykinin. This …

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α1-Antichymotrypsin Present in Therapeutic C1-Inhibitor Products Competes with Selectin–Sialyl LewisX Interaction

Ruchira Engel, Laura Delvasto-Núñez, Dorina Roem, Gerard van Mierlo et autres

Background C1-inhibitor (C1-inh) therapeutics can reduce neutrophil activity in various inflammatory conditions. This ‘novel’ anti-inflammatory effect of C1-inh is attributed to the tetrasaccharide sialyl LewisX (SLeX) present on its N-glycans. Via SLeX, C1-inh is suggested to interact with selectins on inflamed endothelium …

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Human plasma-derived C1 esterase inhibitor concentrate has limited effect on house dust mite-induced allergic lung inflammation in mice

Ingrid Stroo, Jack Yang, Adam A. Anas, J. Daan de Boer et autres

C1 esterase inhibitor (C1-INH) can inhibit multiple pathways (complement, contact-kinin, coagulation, and fibrinolysis) that are all implicated in the pathophysiology of asthma. We explored the effect of human plasma-derived C1-INH on allergic lung inflammation in a house dust mite (HDM) induced asthma …

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Alpha1-Antichymotrypsin Present in Therapeutic C1-Inhibitor Products Competes with Selectin - Sialyl LewisX Interaction

Ruchira Engel, Laura Delvasto Nunez, Dorina Roem, Gerard van Mierlo et autres

Abstract BACKGROUND: The plasma protein C1-inhibitor (C1-inh), belongs to the serpin superfamily and is the major inhibitor of the proteases of the complement and contact phase pathways. Hereditary or acquired deficiency of functional C1-inh results in angioedema episodes in affected individuals due …

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ELISA to measure neutralizing capacity of anti-C1-inhibitor antibodies in plasma of angioedema patients

Ruchira Engel, Irma Rensink, Dorina Roem, Mieke C. Brouwer et autres

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Evaluating the efficacy of subcutaneous C1-esterase inhibitor administration for use in rat models of inflammatory diseases

Reindert W. Emmens, Benno A. Naaijkens, Dorina Roem, Klaas Kramer et autres

CONTEXT: C1-esterase inhibitor (C1-inh) therapy is currently administered to patients with C1-inh deficiency through intravenous injections. The possibility of subcutaneous administration is currently being explored since this would alleviate need for hospitalization and increase mobility and well-being of patients. Recently, it was …

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The effects of continuous venovenous hemofiltration on coagulation activation

Catherine S. C. Bouman, Anne-Cornélie JM de Pont, Joost C.M. Meijers, Kamran Bakhtiari et autres

INTRODUCTION: The mechanism of coagulation activation during continuous venovenous hemofiltration (CVVH) has not yet been elucidated. Insight into the mechanism(s) of hemostatic activation within the extracorporeal circuit could result in a more rational approach to anticoagulation. The aim of the present study …

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