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 …

C3 Deficiencies

D Bitter-Suermann, R Burger - The Third Component of Complement …, 1990 - Springer
The third component of complement is central to both pathways of the complement cascade
and in mediating or initiating the bulk of physiological and often pathological effects of this …

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 …

An inherited deficiency of the third component of complement, C3, in guinea pigs

R Burger, J Gordon, G Stevenson… - European journal of …, 1986 - Wiley Online Library
Hereditary deficiency of the third component of complement, C3, is found very seldom in the
human. C3 deficiency is associated with severe bacterial infections revealing the central role …

Genetic disruption of the murine complement C3 promoter region generates deficient mice with extrahepatic expression of C3 mRNA

A Circolo, G Garnier, W Fukuda, X Wang, T Hidvegi… - …, 1999 - Elsevier
Genetic deficiencies of the complement protein C3 occur naturally in humans and animal
models and have been induced in mice by targeted deletion of the C3 gene. The study of …

The molecular basis for genetic deficiency of the second component of human complement

FS Cole, AS Whitehead, HS Auerbach… - … England Journal of …, 1985 - Mass Medical Soc
Genetic deficiency of the second component of complement (C2) is the most common
complement-deficiency state among Western Europeans and is frequently associated with …

Inherited human complement C3 deficiency. An amino acid substitution in the beta-chain (ASP549 to ASN) impairs C3 secretion.

L Singer, WT Whitehead, H Akama, Y Katz… - Journal of Biological …, 1994 - ASBMB
We recently described a case of hereditary complement C3 deficiency (C3D) in a New
Zealand male who has a small amount of serum C3 (7 micrograms/ml), a normal size 5.2 …

Familial deficiency of C5 in humans: intact but deficient alternative complement pathway activity

RH McLean, G Peter, R Gold, L Guerra, EJ Yunis… - Clinical Immunology and …, 1981 - Elsevier
We report studies of complement-mediated functions in the third family with C5 deficiency.
Four siblings in this black family were found to be C5 deficient (C5-D). All deficient members …

Molecular analysis of the third component of canine complement (C3) and identification of the mutation responsible for hereditary canine C3 deficiency

R Ameratunga, JA Winkelstein, L Brody… - The Journal of …, 1998 - journals.aai.org
Genetically determined deficiency of the third component of complement (C3) in the dog is
characterized by a predisposition to recurrent bacterial infections and to type 1 …

[HTML][HTML] Deficiency of the murine fifth complement component (C5). A 2-base pair gene deletion in a 5'-exon.

RA Wetsel, DT Fleischer, DL Haviland - Journal of Biological Chemistry, 1990 - Elsevier
To ascertain the molecular mechanism that causes murine C5 deficiency, genomic and
cDNA libraries were constructed from mouse liver DNA and mRNA employing the congenic …