Mechanisms of disease -: Alpha1-antitrypsin deficiency -: A model for conformational diseases

被引:291
作者
Carrell, RW
Lomas, DA
机构
[1] Univ Cambridge, Cambridge Inst Med Res, Dept Hematol, Cambridge CB2 2XY, England
[2] Univ Cambridge, Cambridge Inst Med Res, Dept Med, Cambridge CB2 2XY, England
关键词
D O I
10.1056/NEJMra010772
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
[No abstract available]
引用
收藏
页码:45 / 53
页数:9
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共 100 条
  • [1] Abboud R T, 2001, Can Respir J, V8, P81
  • [2] AULAK KS, 1993, J BIOL CHEM, V268, P18088
  • [3] Peripherally administered antibodies against amyloid β-peptide enter the central nervous system and reduce pathology in a mouse model of Alzheimer disease
    Bard, F
    Cannon, C
    Barbour, R
    Burke, RL
    Games, D
    Grajeda, H
    Guido, T
    Hu, K
    Huang, JP
    Johnson-Wood, K
    Khan, K
    Kholodenko, D
    Lee, M
    Lieberburg, I
    Motter, R
    Nguyen, M
    Soriano, F
    Vasquez, N
    Weiss, K
    Welch, B
    Seubert, P
    Schenk, D
    Yednock, T
    [J]. NATURE MEDICINE, 2000, 6 (08) : 916 - 919
  • [4] Antithrombins Wibble and Wobble (T85M/K): Archetypal conformational diseases with in vivo latent - Transition, thrombosis, and heparin activation
    Beauchamp, NJ
    Pike, RN
    Daly, M
    Butler, L
    Makris, M
    Dafforn, TR
    Zhou, A
    Fitton, HL
    Preston, FE
    Peake, IR
    Carrell, RW
    [J]. BLOOD, 1998, 92 (08) : 2696 - 2706
  • [5] Impairment of the ubiquitin-proteasome system by protein aggregation
    Bence, NF
    Sampat, RM
    Kopito, RR
    [J]. SCIENCE, 2001, 292 (5521) : 1552 - 1555
  • [6] LIVER-DISEASE IN ADULTS WITH ALPHA1-ANTITRYPSIN DEFICIENCY
    BERG, NO
    ERIKSSON, S
    [J]. NEW ENGLAND JOURNAL OF MEDICINE, 1972, 287 (25) : 1264 - +
  • [7] Cognitive deficits associated with a recently reported familial neurodegenerative disease - Familial encephalopathy with neuroserpin inclusion bodies
    Bradshaw, CB
    Davis, RL
    Shrimpton, AE
    Holohan, PD
    Rea, CB
    Fieglin, D
    Kent, P
    Collins, GH
    [J]. ARCHIVES OF NEUROLOGY, 2001, 58 (09) : 1429 - 1434
  • [8] BRANTLY M, 1988, AM J MED, V84, P13
  • [9] REPAIR OF THE SECRETION DEFECT IN THE Z-FORM OF ALPHA-1-ANTITRYPSIN BY ADDITION OF A 2ND-MUTATION
    BRANTLY, M
    COURTNEY, M
    CRYSTAL, RG
    [J]. SCIENCE, 1988, 242 (4886) : 1700 - 1702
  • [10] Fibrinogen brescia -: Hepatic endoplasmic reticulum storage and hypofibrinogenemia because of a γ284 Gly→Arg mutation
    Brennan, SO
    Wyatt, J
    Medicina, D
    Callea, F
    George, PM
    [J]. AMERICAN JOURNAL OF PATHOLOGY, 2000, 157 (01) : 189 - 196