Results 1 to 10 of about 721,729 (294)

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
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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
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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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The metabolism of C1 inhibitor and C1q in patients with acquired C1-inhibitor deficiency

Journal of Allergy and Clinical Immunology, 1986
The metabolism of 125I-labeled C1 inhibitor (C1INH) and C1q was studied in five patients with B cell lymphoproliferative disorders, C1INH deficiency, and angioedema. C1INH catabolism was markedly accelerated in these patients. The fractional catabolic rate (FCR) was 0.053 of the plasma pool per hour compared to that of normal subjects (0.025) or ...
J, Melamed, C A, Alper, M, Cicardi, F S, Rosen   +3 more
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C1 inhibitor deficiency: management

Clinical and Experimental Dermatology, 2005
This is the second of two articles on C1 inhibitor deficiency based on a recent UK consensus document covering its diagnosis and management in adults and children. This summary focuses on the management of the disorder including prophylaxis, emergency treatment and special situations such as pregnancy and dental care.
M M, Gompels, R J, Lock
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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
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Structure and Function of C1-Inhibitor

Immunology and Allergy Clinics of North America, 2006
C1-INH belongs to the family of serpins. Structural studies have yielded a clear understanding of the biochemical principle underlying the functional activities of these proteins. Although the crystal structure of C1-INH has yet to be revealed, homology modeling has provided a three-dimensional model of the serpin part of C1-INH.
Ineke G A, Wagenaar-Bos, C Erik, Hack
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Acquired C1 Inhibitor Deficiency

Immunology and Allergy Clinics of North America, 2017
Acquired angioedema due to C1-INH deficiency (C1-INH-AAE) can occur when there are acquired (not inherited) deficiencies of C1-INH. A quantitative or functional C1-INH deficiency with negative family history and low C1q is diagnostic of C1-INH-AAE. The most common conditions associated with C1-INH-AAE are autoimmunity and B-cell lymphoproliferative ...
Iris M, Otani, Aleena, Banerji
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Acute consumption of C1 inhibitor in a patient with acquired C1-inhibitor deficiency syndrome

Journal of Allergy and Clinical Immunology, 1991
Acquired C1-inhibitor (C1 INH) deficiency is usually found in association with an underlying disease that is believed to be responsible for increased C1 INH catabolism, ultimately leading to the development of C1 INH deficiency. We report a remarkable patient with acquired C1 INH deficiency in whom a unique progression of complement- and contact-system
B L, Zuraw, L C, Altman
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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 ...
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