The human complement factor H:: functional roles, genetic variations and disease associations

被引：454
作者：
de Córdoba, SR
Esparza-Gordillo, J
de Jorge, EG
Lopez-Trascasa, M
Sánchez-Corral, P
机构：
[1] Ctr Invest Biol, Dept Inmunol, Madrid 28040, Spain
[2] Hosp Univ La Paz, Unidad Inmunol, Madrid 28046, Spain
来源：
MOLECULAR IMMUNOLOGY | 2004年 / 41卷 / 04期
关键词：
factor H; complement; RCA; hemolytic-uremic-syndrome; HUS;
D O I：
10.1016/j.molimm.2004.02.005
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：
Factor H is an essential regulatory protein that plays a critical role in the homeostasis of the complement system in plasma and in the protection of bystander host cells and tissues from damage by complement activation. Genetic and structural data generated during recent years have been instrumental to delineate the functional domains responsible for these regulatory activities in factor H, which is helping to understand the molecular basis underlying the different pathologies associated to factor H. This review summarises our current knowledge of the role of factor H in health and disease. (C) 2004 Published by Elsevier Ltd.
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收藏
页码：355 / 367
页数：13
相关论文
共 115 条
  • [1] Multipoint quantitative-trait linkage analysis in general pedigrees
    Almasy, L
    Blangero, J
    [J]. AMERICAN JOURNAL OF HUMAN GENETICS, 1998, 62 (05) : 1198 - 1211
  • [2] STRUCTURAL AND FUNCTIONAL-ANALYSIS OF THE COMPLEMENT COMPONENT FACTOR-H WITH THE USE OF DIFFERENT ENZYMES AND MONOCLONAL-ANTIBODIES TO FACTOR-H
    ALSENZ, J
    SCHULZ, TF
    LAMBRIS, JD
    SIM, RB
    DIERICH, MP
    [J]. BIOCHEMICAL JOURNAL, 1985, 232 (03) : 841 - 850
  • [3] Human factor H deficiency - Mutations in framework cysteine residues and block in H protein secretion and intracellular catabolism
    Ault, BH
    Schmidt, BZ
    Fowler, NL
    Kashtan, CE
    Ahmed, AE
    Vogt, BA
    Colten, HR
    [J]. JOURNAL OF BIOLOGICAL CHEMISTRY, 1997, 272 (40) : 25168 - 25175
  • [4] SOLUTION STRUCTURE OF THE 5TH REPEAT OF FACTOR-H - A 2ND EXAMPLE OF THE COMPLEMENT CONTROL PROTEIN MODULE
    BARLOW, PN
    NORMAN, DG
    STEINKASSERER, A
    HORNE, TJ
    PEARCE, J
    DRISCOLL, PC
    SIM, RB
    CAMPBELL, ID
    [J]. BIOCHEMISTRY, 1992, 31 (14) : 3626 - 3634
  • [5] SOLUTION STRUCTURE OF A PAIR OF COMPLEMENT MODULES BY NUCLEAR-MAGNETIC-RESONANCE
    BARLOW, PN
    STEINKASSERER, A
    NORMAN, DG
    KIEFFER, B
    WILES, AP
    SIM, RB
    CAMPBELL, ID
    [J]. JOURNAL OF MOLECULAR BIOLOGY, 1993, 232 (01) : 268 - 284
  • [6] Blackmore TK, 1996, J IMMUNOL, V157, P5422
  • [7] Blackmore TK, 1998, J IMMUNOL, V160, P3342
  • [8] M protein of the group A Streptococcus binds to the seventh short consensus repeat of human complement factor H
    Blackmore, TK
    Fischetti, VA
    Sadlon, TA
    Ward, HM
    Gordon, DL
    [J]. INFECTION AND IMMUNITY, 1998, 66 (04) : 1427 - 1431
  • [9] Complement factor H gene mutation associated with autosomal recessive atypical hemolytic uremic syndrome
    Buddles, MRH
    Donne, RL
    Richards, A
    Goodship, J
    Goodship, THJ
    [J]. AMERICAN JOURNAL OF HUMAN GENETICS, 2000, 66 (05) : 1721 - 1722
  • [10] Complement factor H mutations and gene polymorphisms in haemolytic uraemic syndrome: the C-257T, the A2089G and the G2881T polymorphisms are strongly associated with the disease
    Caprioli, J
    Castelletti, F
    Bucchioni, S
    Bettinaglio, P
    Bresin, E
    Pianetti, G
    Gamba, S
    Brioschi, S
    Daina, E
    Remuzzi, G
    Noris, M
    [J]. HUMAN MOLECULAR GENETICS, 2003, 12 (24) : 3385 - 3395
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