Results 11 to 20 of about 65,236 (304)

The role of C1 inhibitor and complement as acute phase reactants: are we missing the diagnosis of hereditary angioedema?

Allergy, Asthma & Clinical Immunology, 2021
Background C1 inhibitor (C1-INH) and complement 4 (C4) have historically been referred to as positive acute phase reactants, however this has never been evaluated in hereditary angioedema (HAE) patients.
Peter Stepaniuk, Ana-Maria Bosonea, Persia Pourshahnazari, Adrienne Roos, Amin Kanani   +4 more
doaj   +2 more sources

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

mBio, 2021
Complement, contact activation, coagulation, and fibrinolysis are serum protein cascades that need strict regulation to maintain human health. Serum glycoprotein, a C1 inhibitor (C1-INH), is a key regulator (inhibitor) of serine proteases of all the ...
Arun Dhillon, Justin C. Deme, Emily Furlong, Dorina Roem, Ilse Jongerius, Steven Johnson, Susan M. Lea   +6 more
doaj   +2 more sources

Successful long-term prophylaxis of hereditary pregnancy-associated angioedema with plasma-derived C1-inhibitor concentrate: a case report

Медицинская иммунология, 2021
Hereditary angioedema (HAE) is a rare autosomal dominant disease caused by quantitative (type I) or functional (type II) deficiency in C1 esterase inhibitor (C1-INH). It may be caused by new mutations in up to 20% of patients.
D. V. Demina, A. O. Makeeva, L. M. Kudelya, E. V. Novikova, V. A. Kozlov   +4 more
doaj   +2 more sources

Virulence Associated Gene 8 of Bordetella pertussis Enhances Contact System Activity by Inhibiting the Regulatory Function of Complement Regulator C1 Inhibitor

Frontiers in Immunology, 2018
Bordetella pertussis is a Gram-negative bacterium and the causative agent of whooping cough. Whooping cough is currently re-emerging worldwide and, therefore, still poses a continuous global health threat. B. pertussis expresses several virulence factors
Elise S. Hovingh, Elise S. Hovingh, Steven de Maat, Alexandra P. M. Cloherty, Steven Johnson, Elena Pinelli, Coen Maas, Ilse Jongerius, Ilse Jongerius   +8 more
doaj   +2 more sources

Serping1/C1 Inhibitor Affects Cortical Development in a Cell Autonomous and Non-cell Autonomous Manner

Frontiers in Cellular Neuroscience, 2017
Current knowledge regarding regulation of radial neuronal migration is mainly focused on intracellular molecules. Our unbiased screen aimed at identification of non-cell autonomous mechanisms involved in this process detected differential expression of ...
Anna Gorelik, Tamar Sapir, Trent M. Woodruff, Orly Reiner   +3 more
doaj   +2 more sources

Clinical Experience with Berotralstat in Patients with Hereditary Angioedema with Normal C1-Esterase Inhibitor: A Commented Case Series

Journal of Asthma and Allergy
Henry J Kanarek,* Drew Austin Saville Mutschelknaus* Kanarek Allergy, Asthma & Immunology, Leawood, KS, USA*These authors contributed equally to this workCorrespondence: Henry J Kanarek, Tel +1 (913) 451-8555, Fax +1 (913) 327-8553, Email ...
Kanarek HJ, Mutschelknaus DAS
doaj   +2 more sources

Clinical profile and treatment outcomes in patients with hereditary angioedema with normal C1 esterase inhibitor

World Allergy Organization Journal, 2022
Background: Hereditary angioedema (HAE) is often caused by low serum levels or functional deficiency in C1 inhibitor (C1-INH); however, in some cases, C1-INH serum level and function are measured as normal (HAE-nl-C1INH).
Douglas H. Jones, MD, Priya Bansal, MD, Jonathan A. Bernstein, MD, Shahnaz Fatteh, MD, Joseph Harper, PharmD, F. Ida Hsu, MD, Maeve O’Connor, MD, Nami Park, PharmD, Daniel Suez, MD   +8 more
doaj   +1 more source

Recombinant human C1 esterase inhibitor for hereditary angioedema attacks: A European registry

World Allergy Organization Journal, 2021
Background: Hereditary angioedema (HAE) due to C1 esterase inhibitor deficiency (C1-INH-HAE) is characterized by recurrent swelling attacks. A European treatment registry was established to review the adverse event profile and efficacy of recombinant ...
Anna Valerieva, Maria T. Staevska, Vesna Grivcheva-Panovska, Milos Jesenak, Kinga Viktória Kőhalmi, Katarina Hrubiskova, Andrea Zanichelli, Luca Bellizzi, Anurag Relan, Roman Hakl, Henriette Farkas   +10 more
doaj   +1 more source

C1 esterase inhibitor-mediated immunosuppression in COVID-19: Friend or foe?

Clinical Immunology Communications, 2022
From asymptomatic to severe, SARS-CoV-2, causative agent of COVID-19, elicits varying disease severities. Moreover, understanding innate and adaptive immune responses to SARS-CoV-2 is imperative since variants such as Omicron negatively impact adaptive ...
Melissa A. Hausburg, Jason S. Williams, Kaysie L. Banton, Charles W. Mains, Michael Roshon, David Bar-Or   +5 more
doaj   +1 more source

Potential therapeutic benefit of C1-esterase inhibitor in neuromyelitis optica evaluated in vitro and in an experimental rat model. [PDF]

PLoS ONE, 2014
Neuromyelitis optica (NMO) is an autoimmune demyelinating disease of the central nervous system in which binding of anti-aquaporin-4 (AQP4) autoantibodies (NMO-IgG) to astrocytes causes complement-dependent cytotoxicity (CDC) and inflammation resulting ...
Lukmanee Tradtrantip, Nithi Asavapanumas, Puay-Wah Phuan, A S Verkman   +3 more
doaj   +1 more source