MOLECULAR-BASIS FOR THE DEFICIENCY OF COMPLEMENT-1 INHIBITOR IN TYPE-I HEREDITARY ANGIONEUROTIC-EDEMA

被引:65
作者
CICARDI, M
IGARASHI, T
ROSEN, FS
DAVIS, AE
机构
[1] CHILDRENS HOSP MED CTR, DIV IMMUNOL, 300 LONGWOOD AVE, BOSTON, MA 02115 USA
[2] CHILDRENS HOSP MED CTR, DIV NEPHROL, BOSTON, MA 02115 USA
[3] HARVARD UNIV, SCH MED, DEPT PEDIAT, BOSTON, MA 02115 USA
关键词
D O I
10.1172/JCI112873
中图分类号
R-3 [医学研究方法]; R3 [基础医学];
学科分类号
1001 ;
摘要
引用
收藏
页码:698 / 702
页数:5
相关论文
共 26 条
[1]   BIOSYNTHESIS INVITRO OF COMPLEMENT SUBCOMPONENT-C1Q, SUBCOMPONENT-C1S AND SUBCOMPONENT-C1 INHIBITOR BY RESTING AND STIMULATED HUMAN-MONOCYTES [J].
BENSA, JC ;
REBOUL, A ;
COLOMB, MG .
BIOCHEMICAL JOURNAL, 1983, 216 (02) :385-392
[2]   DELETION OF COMPLEMENT C-4 AND STEROID 21-HYDROXYLASE GENES IN THE HLA CLASS-III REGION [J].
CARROLL, MC ;
PALSDOTTIR, A ;
BELT, KT ;
PORTER, RR .
EMBO JOURNAL, 1985, 4 (10) :2547-2552
[3]   ISOLATION OF BIOLOGICALLY-ACTIVE RIBONUCLEIC-ACID FROM SOURCES ENRICHED IN RIBONUCLEASE [J].
CHIRGWIN, JM ;
PRZYBYLA, AE ;
MACDONALD, RJ ;
RUTTER, WJ .
BIOCHEMISTRY, 1979, 18 (24) :5294-5299
[4]   HEREDITARY ANGIOEDEMA - AN APPRAISAL OF 104 CASES [J].
CICARDI, M ;
BERGAMASCHINI, L ;
MARASINI, B ;
BOCCASSINI, G ;
TUCCI, A ;
AGOSTONI, A .
AMERICAN JOURNAL OF THE MEDICAL SCIENCES, 1982, 284 (01) :2-9
[5]   THE MOLECULAR-BASIS FOR GENETIC DEFICIENCY OF THE 2ND COMPONENT OF HUMAN-COMPLEMENT [J].
COLE, FS ;
WHITEHEAD, AS ;
AUERBACH, HS ;
LINT, T ;
ZEITZ, HJ ;
KILBRIDGE, P ;
COLTEN, HR .
NEW ENGLAND JOURNAL OF MEDICINE, 1985, 313 (01) :11-16
[6]   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
[7]   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-+
[8]   VARIABILITY IN PURIFIED DYSFUNCTIONAL C1-INHIBITOR PROTEINS FROM PATIENTS WITH HEREDITARY ANGIONEUROTIC-EDEMA - FUNCTIONAL AND ANALYTICAL GEL STUDIES [J].
DONALDSON, VH ;
HARRISON, RA ;
ROSEN, FS ;
BING, DH ;
KINDNESS, G ;
CANAR, J ;
WAGNER, CJ ;
AWAD, S .
JOURNAL OF CLINICAL INVESTIGATION, 1985, 75 (01) :124-132
[9]   SYNTHESIS OF 2ND COMPONENT OF COMPLEMENT BY LONG-TERM PRIMARY CULTURES OF HUMAN MONOCYTES [J].
EINSTEIN, LP ;
SCHNEEBERGER, EE ;
COLTEN, HR .
JOURNAL OF EXPERIMENTAL MEDICINE, 1976, 143 (01) :114-126
[10]   RESPONSE OF VARIANT HEREDITARY ANGIOEDEMA PHENOTYPES TO DANAZOL THERAPY - GENETIC-IMPLICATIONS [J].
GADEK, JE ;
HOSEA, SW ;
GELFAND, JA ;
FRANK, MM .
JOURNAL OF CLINICAL INVESTIGATION, 1979, 64 (01) :280-286