The role of the complement system in hereditary angioedema

被引：30

作者：

Csuka, Dorottya ^{[1
]}

Veszeli, Nora ^{[1
]}

Varga, Lilian ^{[1
]}

Prohaszka, Zoltan ^{[1
]}

Farkas, Henriette ^{[1
]}

机构：

[1] Semmelweis Univ, Dept Internal Med 3, Kutvolgyi Ut 4, H-1125 Budapest, Hungary

来源：

MOLECULAR IMMUNOLOGY | 2017年 / 89卷

关键词：

Hereditary angioedema; Complement system; C1-inhibitor; Bradykinin; Mutation; C1 INHIBITOR DEFICIENCY; BRADYKININ-MEDIATED ANGIOEDEMA; MOLECULAR-WEIGHT KININOGEN; LECTIN-PATHWAY ACTIVATION; ASSESS SYMPTOM SEVERITY; SERINE-PROTEASE MASP-1; ANGIONEUROTIC-EDEMA; C1-INHIBITOR DEFICIENCY; FACTOR-XII; GENE-MUTATIONS;

D O I：

10.1016/j.molimm.2017.05.020

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

Hereditary angioedema (HAE) is a rare, but potentially life-threatening disorder, characterized by acute, recurring, and self-limiting edematous episodes of the face, extremities, trunk, genitals, upper airways, or the gastrointestinal tract. HAE may be caused by the deficiency of Cl-inhibitor (C1-INH-HAE) but another type of the disease, hereditary angioedema with normal Cl-INH function (nCl-INH-HAE) was also described. The patient population is quite heterogeneous as regards the location, frequency, and severity of edematous attacks, presenting large intra- and inter-individual variation. Here, we review the role of the complement system in the pathomechanism of HAE and also present an overview on the complement parameters having an importance in the diagnosis or in predicting the severity of HAE.

引用

收藏

页码：59 / 68

页数：10

相关论文

共 130 条

[41]

CUGNO M, 1993, J LAB CLIN MED, V121, P38

[42] Angioedema due to acquired C1-inhibitor deficiency: A bridging condition between autoimmunity and lymphoproliferation [J].

Cugno, Massimo ;

Castelli, Roberto ;

Cicardi, Marco .

AUTOIMMUNITY REVIEWS, 2008, 8 (02) :156-159

[43] The effect of sequence variations within the coding region of the C1 inhibitor gene on disease expression and protein function in families with hereditary angio-oedema [J].

Cumming, SA ;

Halsall, DJ ;

Ewan, PW ;

Lomas, DA .

JOURNAL OF MEDICAL GENETICS, 2003, 40 (10) :e114

[44] Acquired angioedema with C1 inhibitor deficiency:: Is the distinction between type I and type II still relevant? [J].

D'Incan, M ;

Tridon, A ;

Ponard, D ;

Dumestre-Pérard, C ;

Ferrier-Le Bouedec, MC ;

Bétail, G ;

Souteyrand, P ;

Caillaud, D .

DERMATOLOGY, 1999, 199 (03) :227-230

[45] HUMAN INHIBITOR OF THE 1ST COMPONENT OF COMPLEMENT, C1 - CHARACTERIZATION OF CDNA CLONES AND LOCALIZATION OF THE GENE TO CHROMOSOME-11 [J].

DAVIS, AE ;

WHITEHEAD, AS ;

HARRISON, RA ;

DAUPHINAIS, A ;

BRUNS, GAP ;

CICARDI, M ;

ROSEN, FS .

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1986, 83 (10) :3161-3165

[46] Plasmin is a natural trigger for bradykinin production in patients with hereditary angioedema with factor XII mutations [J].

de Maat, Steven ;

Bjorkqvist, Jenny ;

Suffritti, Chiara ;

Wiesenekker, Chantal P. ;

Nagtegaal, Willem ;

Koekman, Arnold ;

van Dooremalen, Sanne ;

Pasterkamp, Gerard ;

de Groot, Philip G. ;

Cicardi, Marco ;

Renne, Thomas ;

Maas, Coen .

JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY, 2016, 138 (05) :1414-+

[47] Mannan-Binding Lectin-Associated Serine Protease (MASP)-1 Is Crucial for Lectin Pathway Activation in Human Serum, whereas neither MASP-1 nor MASP-3 Is Required for Alternative Pathway Function [J].

Degn, Soren E. ;

Jensen, Lisbeth ;

Hansen, Annette G. ;

Duman, Duygu ;

Tekin, Mustafa ;

Jensenius, Jens C. ;

Thiel, Steffen .

JOURNAL OF IMMUNOLOGY, 2012, 189 (08) :3957-3969

[48] Cleavage of Kininogen and Subsequent Bradykinin Release by the Complement Component: Mannose-Binding Lectin-Associated Serine Protease (MASP)-1 [J].

Dobo, Jozsef ;

Major, Balazs ;

Kekesi, Katalin A. ;

Szabo, Istvan ;

Megyeri, Marton ;

Hajela, Krishnan ;

Juhasz, Gabor ;

Zavodszky, Peter ;

Gal, Peter .

PLOS ONE, 2011, 6 (05)

[49]

Donaldson V H, 1977, Trans Assoc Am Physicians, V90, P174

[50] A BIOCHEMICAL ABNORMALITY IN HEREDITARY ANGIONEUROTIC EDEMA - ABSENCE OF SERUM INHIBITOR OF C 1-ESTERASE [J].

DONALDSON, VH ;

EVANS, RR .

AMERICAN JOURNAL OF MEDICINE, 1963, 35 (01) :37-+

← 1 2 3 4 5 6 7 8 9 10 →