Results 331 to 340 of about 3,095,271 (376)

Monocyte C1‐inhibitor synthesis in patients with C1‐inhibitor deficiency

European Journal of Clinical Investigation, 1989
Abstract. Monocytes of seven out of eight patients with type 1 C1‐inhibitor (C1‐inh) deficiency (HAE) produced 40% as much C1‐inh as monocytes from normal donors (controls). In contrast, monocytes from three patients with type 2 and three patients with acquired C1‐inh deficiency produced similar amounts of Cl‐inh as controls. Recombinant γ‐interferon (
D F, Lappin, A R, McPhaden, P L, Yap, P E, Carter, G D, Birnie, J E, Fothergill, K, Whaley   +6 more
openaire   +2 more sources

Deletions in SERPING1 Lead to Lower C1 Inhibitor Function: Lower C1 Inhibitor Function Can Predict Disease Severity

International Archives of Allergy and Immunology, 2018
Background: How genotype affects phenotype in hereditary angioedema with C1 inhibitor deficiency (C1-INH-HAE) has not been totally clarified. In this study, we investigated the relationship between different types of mutations and various phenotypic ...
N. Mete Gökmen, O. Gülbahar, H. Onay, Zeynep Peker Koc, Semiha Özgül, T. Köse, A. Gelincik, S. Büyüköztürk, A. Sin   +8 more
semanticscholar   +1 more source

C1-inhibitor deficiency and angioedema

Molecular Immunology, 2001
C1-inhibitor deficiency can be inherited or acquired; both conditions lead to recurrent angioedema that can be life threatening when the larynx is involved (hereditary angioedema, HAE; acquired angioedema, AAE). The genetic defect is due to the heterozygous deficiency of C1-Inh that is transmitted as an autosomal dominant trait.
A. Carugati, E. Pappalardo, L.C. Zingale, M. Cicardi   +3 more
openaire   +3 more sources

C1-inhibitor autoantibodies in SLE

Lupus, 2010
The presence of anti-C1-inhibitor (anti-C1-INH) autoantibodies is a hallmark of acquired C1-inhibitor deficiency. However, only scarce data are available on their prevalence, diagnostic value, and/or significance in systemic lupus erythematosus (SLE).
T, Mészáros, G, Füst, H, Farkas, L, Jakab, G, Temesszentandrási, G, Nagy, E, Kiss, P, Gergely, M, Zeher, Z, Griger, L, Czirják, R, Hóbor, A, Haris, K, Polner, L, Varga   +14 more
openaire   +2 more sources

C1 inhibitor: molecular and clinical aspects

Springer Seminars in Immunopathology, 2005
C1 inhibitor (C1-INH) is a serine protease inhibitor (serpins) that inactivates several different proteases in the complement, contact, coagulation, and fibrinolytic systems. By its C-terminal part (serpin domain), characterized by three beta-sheets and an exposed mobile reactive loop, C1-INH binds, and blocks the activity of its target proteases.
M. Cicardi, L. Zingale, A. Zanichelli, E. Pappalardo, CICARDI, BENEDETTA   +4 more
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C1 Inhibitor Autoantibodies

2014
Autoantibodies to C1 inhibitor (C1-INH) bind to epitopes on the reactive center of the C1-INH molecule. As a consequence of this binding, C1-INH is converted into an inactive substrate that can be cleaved by proteases. C1-INH is a serine protease inhibitor that plays a role in the complement, contact, fibrinolytic, and coagulation cascades ...
Zanichelli, A, Suffritti, C, Cicardi, M, PERRICONE, ROBERTO   +3 more
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[6] C1 Inhibitor

1993
Publisher Summary This chapter discusses C1 inhibitor (C1 INH). C1 INH is a member of the serine proteinase inhibitor (serpin) superfamily. C1 INH is the only physiologically important plasma inhibitor of activated Clr and Cls, and it provides a major portion of the plasma inhibitory capacity toward kallikrein and factor XIIa, but probably is not ...
Alvin E. Davis, Kulwant S. Aulak, Kamyar Zahedi, John J. Bissler, Richard A. Harrison   +4 more
openaire   +1 more source

C1 Inhibitor: Quantification and Purification

2013
C1 inhibitor is a multipotent serpin capable of inhibiting the classical and the lectin pathways of complement, the fibrinolytic system, and contact/kinin system of coagulation. Deficiency of C1 inhibitor manifest as hereditary angioedema (HAE), an autosomal dominant hereditary disease.
Varga, Lilian, Dobó, József
openaire   +2 more sources

[C1-inhibitor].

Nihon rinsho. Japanese journal of clinical medicine, 2000
Ranol Zahedi, Alvin E. Davis
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Biological effects of C1 inhibitor

Drug News & Perspectives, 2004
C1 inhibitor is a serine proteinase inhibitor (serpin) that regulates activation of both the complement and contact systems. Regulation of complement system activation takes place through inactivation of the classical pathway proteases, C1r and C1s, the lectin pathway protease, MASP2, and perhaps via inhibition of alternative pathway activation by ...
openaire   +2 more sources