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Classical pathway deficiencies - A short analytical review.

Truedsson, Lennart LU (2015) In Molecular Immunology 68(1). p.14-19
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... (More)
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. (Less)
Please use this url to cite or link to this publication:
author
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
Molecular Immunology
volume
68
issue
1
pages
14 - 19
publisher
Pergamon
external identifiers
  • pmid:26038300
  • wos:000363822400004
  • scopus:84952637056
ISSN
1872-9142
DOI
10.1016/j.molimm.2015.05.007
language
English
LU publication?
yes
id
576812d4-118c-424f-9142-ed0b8c4b2412 (old id 7508439)
alternative location
http://www.ncbi.nlm.nih.gov/pubmed/26038300?dopt=Abstract
date added to LUP
2015-07-06 02:06:53
date last changed
2017-09-24 03:06:59
@article{576812d4-118c-424f-9142-ed0b8c4b2412,
  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.},
  author       = {Truedsson, Lennart},
  issn         = {1872-9142},
  language     = {eng},
  number       = {1},
  pages        = {14--19},
  publisher    = {Pergamon},
  series       = {Molecular Immunology},
  title        = {Classical pathway deficiencies - A short analytical review.},
  url          = {http://dx.doi.org/10.1016/j.molimm.2015.05.007},
  volume       = {68},
  year         = {2015},
}