Clinical aspects and molecular basis of primary deficiencies of complement component C3 and its regulatory proteins factor I and factor H
E S. Reis, DA Falcao, L Isaac - Scandinavian journal of …, 2006 - Wiley Online Library
The complement system participates in both innate and acquired immune responses.
Deficiencies in any of the protein components of this system are generally uncommon and …
Deficiencies in any of the protein components of this system are generally uncommon and …
Complement C3 deficiency: human, animal, and experimental models
L Singer, HR Colten, RA Wetsel - Pathobiology, 1994 - karger.com
The third complement component (C3) is a multifunctional glycoprotein that interacts with
numerous serum proteins, cell surface receptors, and membrane-associated regulatory …
numerous serum proteins, cell surface receptors, and membrane-associated regulatory …
Deficiency of human complement protein C4 due to identical frameshift mutations in the C4A and C4B genes
ML Lokki, A Circolo, P Ahokas, KL Rupert… - The Journal of …, 1999 - journals.aai.org
The complement protein C4, encoded by two genes (C4A and C4B) on chromosome 6p, is
the most polymorphic among the MHC III gene products. We investigated the molecular …
the most polymorphic among the MHC III gene products. We investigated the molecular …
The molecular basis of complete complement C4A and C4B deficiencies in a systemic lupus erythematosus patient with homozygous C4A and C4B mutant genes
KL Rupert, JM Moulds, Y Yang, FC Arnett… - The Journal of …, 2002 - journals.aai.org
The disease course of a complete C4-deficient patient in the US was followed for 18 years.
The patient experienced multiple episodes of infection, and he was diagnosed with systemic …
The patient experienced multiple episodes of infection, and he was diagnosed with systemic …
Complete complement components C4A and C4B deficiencies in human kidney diseases and systemic lupus erythematosus
Y Yang, K Lhotta, EK Chung, P Eder… - The Journal of …, 2004 - journals.aai.org
Although a heterozygous deficiency of either complement component C4A or C4B is
common, and each has a frequency of∼ 20% in a Caucasian population, complete …
common, and each has a frequency of∼ 20% in a Caucasian population, complete …
[HTML][HTML] Molecular heterogeneity of C2 deficiency
CA Johnson, P Densen, RA Wetsel… - … England Journal of …, 1992 - Mass Medical Soc
The most common of the complement deficiencies in populations of European origin is a
genetic deficiency of the second component of complement (C2). 1 Rheumatologic …
genetic deficiency of the second component of complement (C2). 1 Rheumatologic …
Molecular basis for complement component 6 (C6) deficiency in rats and mice
D Bhole, GL Stahl - Immunobiology, 2004 - Elsevier
Complement component C6 is a part of the lytic membrane attack complex formed during
complement activation. Animal modeling to define the role of C5a vs. C5b-9 in human …
complement activation. Animal modeling to define the role of C5a vs. C5b-9 in human …
Structural insights into the central complement component C3
BJC Janssen, P Gros - Molecular immunology, 2007 - Elsevier
C3 is a central protein of the complement system, which is important to immune defense and
provides a link between innate and adaptive immunity. Three pathways of complement …
provides a link between innate and adaptive immunity. Three pathways of complement …
Hereditary deficiency of C3 in animals and humans
M Botto, MJ Walport - International Reviews of Immunology, 1993 - Taylor & Francis
Inherited deficiency of complement C3 has been described in guinea pigs, dogs and 20
humans. Homozygous deficiency of C3 is associated with recurrent pyogenic infections by …
humans. Homozygous deficiency of C3 is associated with recurrent pyogenic infections by …
Complement component C3–The “Swiss Army Knife” of innate immunity and host defense
As a preformed defense system, complement faces a delicate challenge in providing an
immediate, forceful response to pathogens even at first encounter, while sparing host cells in …
immediate, forceful response to pathogens even at first encounter, while sparing host cells in …
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