A 30-year perspective on alpha(1)-antitrypsin deficiency

被引:53
作者
Eriksson, S
机构
[1] Department of Medicine, University of Lund, University Hospital of Malmö, Malmö
[2] Department of Medicine, University Hospital of Malmö
关键词
alpha(1)-antitrypsin deficiency; emphysema; endoplasmic reticulum storage diseases; serpins; vasculitic syndromes;
D O I
10.1378/chest.110.6_Supplement.237S
中图分类号
R4 [临床医学];
学科分类号
1002 ; 100602 ;
摘要
The early history of alpha(1)-antitrypsin deficiency, in the years between 1962 and 1965, and its impact on the understanding of emphysema abnormalities were reviewed by me in 1989. This report discusses the disease spectrum (including emphysema, liver, and vasculitic diseases) and focuses on the variable clinical expression of the deficiency state. Some new findings that may be relevant to the function of alpha(1)-antitrypsin are also considered.
引用
收藏
页码:S237 / S242
页数:6
相关论文
共 29 条
[1]  
BLANK CA, 1994, ANN ALLERGY, V72, P105
[2]   STRUCTURE AND VARIATION OF HUMAN ALPHA-1-ANTITRYPSIN [J].
CARRELL, RW ;
JEPPSSON, JO ;
LAURELL, CB ;
BRENNAN, SO ;
OWEN, MC ;
VAUGHAN, L ;
BOSWELL, DR .
NATURE, 1982, 298 (5872) :329-334
[3]   THE SERPINS - EVOLUTION AND ADAPTATION IN A FAMILY OF PROTEASE INHIBITORS [J].
CARRELL, RW ;
PEMBERTON, PA ;
BOSWELL, DR .
COLD SPRING HARBOR SYMPOSIA ON QUANTITATIVE BIOLOGY, 1987, 52 :527-535
[4]  
CHANDRA RK, 1987, CANCER RES, V47, P1179
[5]   ALPHA-1-ANTITRYPSIN DEFICIENCY, EMPHYSEMA, AND LIVER-DISEASE - GENETIC-BASIS AND STRATEGIES FOR THERAPY [J].
CRYSTAL, RG .
JOURNAL OF CLINICAL INVESTIGATION, 1990, 85 (05) :1343-1352
[6]   STRONG LINK BETWEEN THE ALPHA(1)-ANTITRYPSIN PIZ ALLELE AND WEGENERS GRANULOMATOSIS [J].
ELZOUKI, ANY ;
SEGELMARK, M ;
WIESLANDER, J ;
ERIKSSON, S .
JOURNAL OF INTERNAL MEDICINE, 1994, 236 (05) :543-548
[8]   THE PUTATIVE ROLE OF ALPHA-1-ANTITRYPSIN IN THE DISAGGREGATION OF AMYLOID-LAMBDA FIBRILS [J].
ERIKSSON, S ;
JANCIAUSKIENE, S ;
MERLINI, G .
JOURNAL OF INTERNAL MEDICINE, 1995, 237 (02) :143-149
[9]   ALPHA(1)-ANTICHYMOTRYPSIN REGULATES ALZHEIMER BETA-AMYLOID PEPTIDE FIBRIL FORMATION [J].
ERIKSSON, S ;
JANCIAUSKIENE, S ;
LANNFELT, L .
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1995, 92 (06) :2313-2317
[10]   ALPHA-1-PROTEASE INHIBITOR IN BRONCHIAL-ASTHMA - PHENOTYPES AND BIOCHEMICAL CHARACTERISTICS [J].
GAILLARD, MC ;
KILROESMITH, TA ;
NOGUEIRA, C ;
DUNN, D ;
JENKINS, T ;
FINE, B ;
KALLENBACH, J .
AMERICAN REVIEW OF RESPIRATORY DISEASE, 1992, 145 (06) :1311-1315