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Behavior in vivo of normal and dysfunctional C1 inhibitor in normal subjects and patients with hereditary angioneurotic edema.

M Quastel, … , C A Alper, F S Rosen

M Quastel, … , C A Alper, F S Rosen

Published April 1, 1983
Citation Information: J Clin Invest. 1983;71(4):1041-1046. https://doi.org/10.1172/JCI110831.

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Citations to this article (96)

Title and authors	Publication	Year
Genetic variants leading to urticaria and angioedema and associated biomarkers Lyons JJ, Farkas H, Germenis AE, Rijavec M, Smith TD, Valent P	The journal of allergy and clinical immunology. In practice	2023
Sugar Matters: Improving In Vivo Clearance Rate of Highly Glycosylated Recombinant Plasma Proteins for Therapeutic Use S Zeerleder, R Engel, T Zhang, D Roem, G van Mierlo, I Wagenaar-Bos, S van Ham, M Wuhrer, D Wouters, I Jongerius	Pharmaceuticals (Basel, Switzerland)	2021
C1 Inhibitor Activity and Angioedema Attacks in Patients with Hereditary Angioedema AP Kaplan, D Pawaskar, J Chiao	The Journal of Allergy and Clinical Immunology: In Practice	2019
The Role of Complement in Hereditary Angioedema M Levi, DM Cohn	Transfusion Medicine Reviews	2019
Intermittent C1-Inhibitor Deficiency Associated with Recessive Inheritance: Functional and Structural Insight S Caccia, C Suffritti, T Carzaniga, R Berardelli, S Berra, V Martorana, A Fra, C Drouet, M Cicardi	Scientific Reports	2018
HAE Pathophysiology and Underlying Mechanisms BL Zuraw, SC Christiansen	Clinical Reviews in Allergy & Immunology	2016
Hereditary and acquired C1-inhibitor-dependent angioedema: from pathophysiology to treatment S Zeerleder, M Levi	Annals of Medicine	2016
Genetics of Hereditary Angioedema Revisited AE Germenis, M Speletas	Clinical Reviews in Allergy & Immunology	2016
How we manage persons with hereditary angioedema BL Zuraw, SC Christiansen	British Journal of Haematology	2016
Phase II study results of a replacement therapy for hereditary angioedema with subcutaneous C1-inhibitor concentrate BL Zuraw, M Cicardi, HJ Longhurst, JA Bernstein, HH Li, M Magerl, I Martinez-Saguer, SM Rehman, P Staubach, H Feuersenger, R Parasrampuria, J Sidhu, J Edelman, T Craig	Allergy	2015
Pathophysiology of Hereditary Angioedema S Caccia, C Suffritti, M Cicardi	Pediatric Allergy, Immunology, and Pulmonology	2014
Current update on cellular and molecular mechanisms of hereditary angioedema HH Walford, BL Zuraw	Annals of Allergy, Asthma & Immunology	2014
Identification and characterization of a novel splice site mutation in the SERPING1 gene in a family with hereditary angioedema R Colobran, S Lois, X la Cruz, R Pujol-Borrell, M Hernández-González, M Guilarte	Clinical Immunology	2014
Stiehm's Immune Deficiencies H Yarmohammadi	Stiehm's Immune Deficiencies	2014
Alterations of coagulation and fibrinolysis in patients with angioedema due to C1-inhibitor deficiency : Coagulation and fibrinolysis in angioedema M Geffen, M Cugno, P Lap, A Loof, M Cicardi, W Heerde	Clinical & Experimental Immunology	2012
Production of Plasma Proteins for Therapeutic Use: Bertolini/Plasma Proteins S Chtourou, M Poulle	Production of Plasma Proteins for Therapeutic Use: Bertolini/Plasma Proteins	2012
Current and Emerging Options for Hereditary Angioedema Management AP Kaplan	Current and Emerging Options for Hereditary Angioedema Management	2012
Alterations of coagulation and fibrinolysis in patients with angioedema due to C1-inhibitor deficiency M Geffen, M Cugno, P Lap, A Loof, M Cicardi, W Heerde	Clinical & Experimental Immunology	2012
Therapeutic Approaches in Hereditary Angioedema SA Antoniu	Clinical Reviews in Allergy & Immunology	2011
A single nucleotide deletion at the C1 inhibitor gene as the cause of hereditary angioedema: insights from a Brazilian family MF Ferraro, AS Moreno, EC Castelli, EA Donadi, MS Palma, HA Arcuri, AP Lange, K Bork, W Sarti, LK Arruda	Allergy	2011
Harper's Textbook of Pediatric Dermatology: Irvine/Harper's Textbook of Pediatric Dermatology R Happle	Harper's Textbook of Pediatric Dermatology: Irvine/Harper's Textbook of Pediatric Dermatology	2011
A single nucleotide deletion at the C1 inhibitor gene as the cause of hereditary angioedema: insights from a Brazilian family: Hereditary angioedema: insights from a Brazilian family MF Ferraro, AS Moreno, EC Castelli, EA Donadi, MS Palma, HA Arcuri, AP Lange, K Bork, W Sarti, LK Arruda	Allergy	2011
C1 Esterase Inhibitor (Human) for the Treatment of Acute Hereditary Angioedema SO Valle, AT França, RA Campos, AS Grumach	Clinical medicine. Blood disorders	2011
THE GENETIC AND CLINICAL ASPECTS OF HAE, NEWPOSSIBILITIES OF THE DISEASE EXACERBATIONS TREATMENT TV Latysheva, AV Polyakov, AV Dmitrieva, EN Medunitsyna, TV Latysheva, AV Polyakov, AV Dmitrieva, EN Medunitsyna		2011
The Pathophysiology of Hereditary Angioedema BL Zuraw	World Allergy Organization Journal	2010
Hereditary Angioedema in Childhood: An Approach to Management DG Ebo, MM Verweij, KJ Knop, MM Hagendorens, CH Bridts, LS Clerck, WJ Stevens	Pediatric Drugs	2010
Hereditary Angioedema in Childhood DG Ebo, MM Verweij, KJ de Knop, MM Hagendorens, CH Bridts, LS de Clerck, WJ Stevens	Pediatric Drugs	2010
Pharmacokinetic analysis of human plasma-derived pasteurized C1-inhibitor concentrate in adults and children with hereditary angioedema: a prospective study I Martinez-Saguer, E Rusicke, E Aygören-Pürsün, N Hentig, T Klingebiel, W Kreuz	Transfusion	2010
Hereditary Angioedema: Current and Emerging Treatment Options JH Levy, DJ Freiberger, J Roback	Anesthesia & Analgesia	2010
Hereditary Angioedema in Childhood: An Approach to Management DG Ebo, MM Verweij, KJ de Knop, MM Hagendorens, CH Bridts, LS de Clerck, WJ Stevens	Pediatric Drugs	2010
The Pathophysiology of Hereditary Angioedema BL Zuraw	World Allergy Organization Journal	2010
C1-inhibitor deficiency and angioedema: molecular mechanisms and clinical progress M Cugno, A Zanichelli, F Foieni, S Caccia, M Cicardi	Trends in Molecular Medicine	2009
Middleton's Allergy: Principles and Practice RP Schleimer	Middleton's Allergy: Principles and Practice	2009
Hereditary angioedema: a current state-of-the-art review, III: mechanisms of hereditary angioedema AE Davis	Annals of Allergy Asthma & Immunology	2008
Functional C1-Inhibitor diagnostics in hereditary angioedema: Assay evaluation and recommendations IG Wagenaar-Bos, C Drouet, E Aygören-Pursun, K Bork, C Bucher, A Bygum, H Farkas, G Fust, H Gregorek, CE Hack, A Hickey, HI Joller-Jemelka, M Kapusta, W Kreuz, H Longhurst, M Lopez-Trascasa, K Madalinski, J Naskalski, E Nieuwenhuys, D Ponard, L Truedsson, L Varga, EW Nielsen, E Wagner, L Zingale, M Cicardi, SM van Ham	Journal of Immunological Methods	2008
New treatments addressing the pathophysiology of hereditary angioedema AE Davis	Clinical and Molecular Allergy	2008
Angioedema AP Kaplan	World Allergy Organization Journal	2008
The use of plasma-derived C1 inhibitor in the treatment of hereditary angioedema M Cicardi, LC Zingale, A Zanichelli, DL Deliliers, S Caccia	Expert Opinion on Pharmacotherapy	2007
Complement before molecular biology P Lachmann	Molecular Immunology	2006
Mechanism of Angioedema in First Complement Component Inhibitor Deficiency AE Davis	Immunology and Allergy Clinics of North America	2006
Hereditary angioedema: causes, manifestations and treatment HJ Longhurst, K Bork	BRIT J HOSP MED	2006
The pathophysiology of hereditary angioedema AE Davis	Clinical Immunology	2005
Angioedema AP Kaplan, MW Greaves	Journal of the American Academy of Dermatology	2005
Hereditary and acquired angioedema: Problems and progress: Proceedings of the third C1 esterase inhibitor deficiency workshop and beyond A Agostoni, E Aygorenpursun, K Binkley, A Blanch, K Bork, L Bouillet, C Bucher, A Castaldo, M Cicardi, A DAVISIII	Journal of Allergy and Clinical Immunology	2004
Increased expression of C1-inhibitor mRNA in patients with hereditary angioedema treated with Danazol E Pappalardo, LC Zingale, M Cicardi	Immunology Letters	2003
A multicentre evaluation of the diagnostic efficiency of serological investigations for C1 inhibitor deficiency MM Gompels, RJ Lock, JE Morgan, J Osborne, A Brown, PF Virgo	Journal of clinical pathology	2002
Angioedema E Frigas, UC Nzeako	Clinical Reviews in Allergy & Immunology	2002
A Review of the Reported Defects in the Human C1 Esterase Inhibitor Gene Producing Hereditary Angioedema Including Four New Mutations B Bowen, JJ Hawk, S Sibunka, S Hovick, JM Weiler	Clinical Immunology	2001
C1-inhibitor: an anti-inflammatory reagent with therapeutic potential M Kirschfink, TE Mollnes	Expert Opinion on Pharmacotherapy	2001
Unmasking of acquired autoimmune C1-inhibitor deficiency by an angiotensin-converting enzyme inhibitor GI Kleiner, P Giclas, G Stadtmauer, C Cunningham-Rundles	Annals of Allergy Asthma & Immunology	2001
Dosage, Deletions and Dominance: Simple Models of the Evolution of Gene Expression LD Hurst, JP Randerson	Journal of Theoretical Biology	2000
Angioedema associated with C1 inhibitor deficiency J Laurent, MT Guinnepain	Clinical Reviews in Allergy & Immunology	1999
Regulation of C1 Inhibitor Synthesis AE Prada, K Zahedi, AE Davis	Immunobiology	1998
C1q and Systemic Lupus Erythematosus MJ Walport, KA Davies, M Botto	Immunobiology	1998
ACE Inhibitor???Induced Angioedema: Incidence, Prevention and Management W Vleeming, JG van Amsterdam, BH Ch., DJ de Wildt	Drug Safety	1998
Les angio-œdèmes par déficit en C1 inhibiteur J Laurent, MT Guinnepain	Revue Française d'Allergologie et d'Immunologie Clinique	1997
The Kinin System A Schmaier	The Kinin System	1997
Urticaria, Angioedema, and Autoimmunity BL Zuraw	Clinics in Laboratory Medicine	1997
Review M Epstein	Renal Failure	1996
Crucial residues in the carboxy-terminal end of C1 inhibitor revealed by pathogenic mutants impaired in secretion or function E Verpy, E Couture-Tosi, E Eldering, M Lopez-Trascasa, P Späth, T Meo, M Tosi	Journal of Clinical Investigation	1995
Protein S deficiency in lupus erythematosus secondary to hereditary angio-oedema W Perkins, G Stables, R Lever	British Journal of Dermatology	1994
Additional Tests of Interest to the Dermatologist KL Farmer	Dermatologic Clinics	1994
Transinhibition of C1 inhibitor synthesis in type I hereditary angioneurotic edema J Kramer, FS Rosen, HR Colten, K Rajczy, RC Strunk	Journal of Clinical Investigation	1993
C1-Inhibitor and Its Genetic Alterations in Hereditary Angioneurotic Edema VH Donaldson	International Reviews of Immunology	1993
C1-inhibitor substitution therapy in septic shock and in the vascular leak syndrome induced by high doses of interleukin-2 CE Hack, AC Ogilvie, B Eisele, AJ Eerenberg, J Wagstaff, LG Thijs	Intensive Care Medicine	1993
Autoimmune C1 inhibitor deficiency: Report of eight patients M Cicardi, G Bisiani, M Cugno, P Spath, A Agostoni	The American Journal of Medicine	1993
Activators and Inhibitors of Complement RB Sim		1993
Complement in Health and Disease K Whaley, M Loos, JM Weiler		1993
Antithrombin III, C1 inhibitor, methylglyoxal, and polymorphonuclear leukocytes in the development of vascular complications in diabetes mellitus YA Aleksandrovskii	Thrombosis Research	1992
The Complement System A Agostoni, M Cicardi, M Gardinali, L Bergamaschini	International journal of immunopathology and pharmacology	1992
Synthesis of C1 inhibitor in fibroblasts from patients with type I and type II hereditary angioneurotic edema J Kramer, Y Katz, FS Rosen, AE 3rd, RC Strunk	Journal of Clinical Investigation	1991
The Urticarias 1990 AK Black, M Greaves, R Champion, R Pye	British Journal of Dermatology	1991
Genetic defects of the complement system FS Rosen	Clinical Immunology and Immunopathology	1991
Plasma levels of C1- inhibitor complexes and cleaved C1- inhibitor in patients with hereditary angioneurotic edema M Cugno, J Nuijens, E Hack, A Eerenberg, D Frangi, A Agostoni, M Cicardi	Journal of Clinical Investigation	1990
Identification of a new P1 residue mutation (444Arg→Ser) in a dysfunctional C1 inhibitor protein contained in a type II hereditary angioedema plasma KS Aulak, M Cicardi, RA Harrison	FEBS Letters	1990
Type I C1 inhibitor deficiency with a small messenger RNA resulting from deletion of one exon T Ariga, T Igarashi, N Ramesh, R Parad, M Cicardi, AE 3rd	Journal of Clinical Investigation	1989
Monocyte C1-inhibitor synthesis in patients with C1-inhibitor deficiency DF Lappin, AR McPHADEN, PL Yap, PE Carter, GD Birnie, JE Fothergill, K Whaley	European Journal of Clinical Investigation	1989
Pathways of kinin formation and role in allergic diseases AP Kaplan, M Silverberg, B Ghebrehiwet, P Atkins, B Zweiman	Clinical Immunology and Immunopathology	1989
Kinins V K Abe, H Moriya, S Fujii		1989
CpG Mutations in the Reactive Site of Human C 1¯ Inhibitor K Skriver, E Radziejewska, JA Silbermann, VH Donaldson, SC Bock	The Journal of biological chemistry	1989
A common neoepitope is created when the reactive center of C1-inhibitor is cleaved by plasma kallikrein, activated factor XII fragment, C1 esterase, or neutrophil elastase A Agostini, PA Patston, V Marottoli, S Carrel, PC Harpel, M Schapira	Journal of Clinical Investigation	1988
Molecular basis for the deficiency of complement 1 inhibitor in type I hereditary angioneurotic edema M Cicardi, T Igarashi, FS Rosen, AE 3rd	Journal of Clinical Investigation	1987
Inherited deficiencies of complement components in man CA Alper	Immunology Letters	1987
Metabolism of C4 and linkage analysis in a kindred with hereditary incomplete C4 deficiency JJ Wisnieski, MH Nathanson, JE Anderson, AE Davis, CA Alper, GB Naff	Arthritis & Rheumatism	1987
Altered C1 Inhibitor Genes in Type I Hereditary Angioedema D Stoppa-Lyonnet, M Tosi, J Laurent, A Sobel, G Lagrue, T Meo	New England Journal of Medicine	1987
Autoantibody-Mediated Acquired Deficiency of C1 Inhibitor J Alsenz, K Bork, M Loos	New England Journal of Medicine	1987
Genetics of the Complement System CA Alper	Annals of the New York Academy of Sciences	1986
Complement D Vergani	Diabetic Medicine	1986
Decreased synthesis of serum carboxypeptidase N (SCPN) in familial SCPN deficiency KP Mathews, JG Curd, TE Hugli	Journal of Clinical Immunology	1986
Variability in purified dysfunctional C1(-)-inhibitor proteins from patients with hereditary angioneurotic edema. Functional and analytical gel studies VH Donaldson, RA Harrison, FS Rosen, DH Bing, G Kindness, J Canar, CJ Wagner, S Awad	Journal of Clinical Investigation	1985
Urticaria and angioedema BA Burrall, AC Huntley	Clinical Reviews in Allergy	1985
Simultaneous turnover of normal and dysfunctional C1 inhibitor as a probe of in vivo activation of C1 and contact activatable proteases P Woo, PJ Lachmann, RA Harrison, N Amos, C Cooper, FS Rosen	Clinical & Experimental Immunology	1985
Acquired C1-Inhibitor Deficiency Associated with Antiidiotypic Antibody to Monoclonal Immunoglobulins RS Geha, I Quinti, KF Austen, M Cicardi, A Sheffer, FS Rosen	New England Journal of Medicine	1985
Inherited deficiencies of complement proteins in man CA Alper, FS Rosen	Springer Seminars in Immunopathology	1984
Eczematous and immunologic diseases I Gigli, JM Hanifin, SI Katz, TT Provost, NA Soter	Journal of the American Academy of Dermatology	1984
Hereditary Angioedema CM Brickman, SW Hosea	International Journal of Dermatology	1983

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