Abstract:
:Deficiencies in the classical pathway of complement activation have some common features but show also great differences. Deficiencies of each of the components (C1q, C1s, C1r, C4 and C2) imply increased susceptibility to bacterial infections. They are also associated with increased risk to develop systemic lupus erythematosus where deficiency of C1q is strongly associated to the disease while C4 less and C2 much less. Deficiency of C1q affects only activation of the classical pathway while deficiency of C4 and C2 also prevent activation of the lectin pathway. Bypass mechanisms may result in complement activation also in absence of C2 but not in absence of C1q or C4. The genes for C2 and C4 isotypes are closely located within the MHC class III region on chromosome 6p and the genes for the 3 C1q chains are on chromosome 1p. Deficiencies of C1q and of C4 show genetic heterogeneity while deficiency of C2 in the great majority of cases is caused by a specific deletion. The production of C4 and C2 is mainly by the hepatocytes in the liver while C1q is produced by monocytic bone marrow derived cells. This has implications for the possibility to treat the deficiency and hematopoietic stem cell transplantation has been tried in C1q deficiency.
journal_name
Mol Immunoljournal_title
Molecular immunologyauthors
Truedsson Ldoi
10.1016/j.molimm.2015.05.007subject
Has Abstractpub_date
2015-11-01 00:00:00pages
14-9issue
1eissn
0161-5890issn
1872-9142pii
S0161-5890(15)00397-1journal_volume
68pub_type
杂志文章,评审abstract::The aim of the study was to explore whether fever-range hyperthermia (FRH) might enhance the anticancer and immunoregulatory activities of protein-bound polysaccharides (PBP), a class of fungus derived immunomodifiers used in the cancer adjuvant therapy. Blood lymphocytes and breast cancer cells (MCF-7) were cultured ...
journal_title:Molecular immunology
pub_type: 杂志文章
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journal_title:Molecular immunology
pub_type: 杂志文章
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pub_type: 杂志文章
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pub_type: 杂志文章
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pub_type: 杂志文章
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更新日期:2017-12-01 00:00:00
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journal_title:Molecular immunology
pub_type: 杂志文章,评审
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journal_title:Molecular immunology
pub_type: 杂志文章,评审
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更新日期:2019-10-01 00:00:00
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journal_title:Molecular immunology
pub_type: 杂志文章
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更新日期:2012-07-01 00:00:00
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pub_type: 杂志文章
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更新日期:1998-10-01 00:00:00
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pub_type: 杂志文章
doi:10.1016/j.molimm.2010.08.003
更新日期:2010-11-01 00:00:00
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journal_title:Molecular immunology
pub_type: 杂志文章
doi:10.1016/j.molimm.2016.09.004
更新日期:2016-10-01 00:00:00
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pub_type: 杂志文章
doi:10.1016/j.molimm.2012.02.110
更新日期:2012-05-01 00:00:00
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pub_type: 杂志文章
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pub_type: 杂志文章
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journal_title:Molecular immunology
pub_type: 杂志文章,评审
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journal_title:Molecular immunology
pub_type: 杂志文章
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更新日期:2011-07-01 00:00:00
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journal_title:Molecular immunology
pub_type: 杂志文章
doi:10.1016/j.molimm.2007.10.037
更新日期:2008-04-01 00:00:00
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pub_type: 杂志文章
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更新日期:1993-11-01 00:00:00
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pub_type: 杂志文章
doi:10.1016/0161-5890(93)90009-z
更新日期:1993-07-01 00:00:00
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journal_title:Molecular immunology
pub_type: 杂志文章
doi:10.1016/0161-5890(95)00059-n
更新日期:1995-09-01 00:00:00
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pub_type: 杂志文章
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更新日期:1990-01-01 00:00:00