Complement Component 3

Alternative Names

  • C3
  • C3a, Included
  • C3b, Included
  • C3c, Included
  • C3d, Included
  • Acylation-Stimulating Protein, Included
  • ASP, Included
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OMIM Number

120700

NCBI Gene ID

718

Uniprot ID

P01024

Length

42,947 bases

No. of Exons

41

No. of isoforms

1

Protein Name

Complement C3

Molecular Mass

187148 Da

Amino Acid Count

1663

Genomic Location

chr19:6,677,704-6,720,650

Gene Map Locus
19p13.3

Description

The complement component 3 (c3) is a protein factor, which is part of the complement system, a cascade of serum proteins that play a role in the development of inflammatory reactions. It plays a major part in both the classical and alternative complement pathways. The C3 convertase cleaves C3 into C3a and C3b; both of which play important roles in the cascade. C3a induces vasodilation, increases capillary permeability, promotes phagocyte chemotaxis, and most importantly, is an anaphylatoxin, which acts as a chemotactic attractant for the induction of neutrophilic release of antimicrobial proteases and oxygen radicals. C3b, on the other hand, acts as an opsonin for antigen-antibody complexes, and forms immune aggregates.

C3 levels in the serum and other body fluids are used in the identification of several disease states. Increased C3 levels are seen in conditions such as cancer, rheumatoid arthritis, and ulcerative colitis, whereas reduced levels are observed in systemic lupus erythematosus, bacterial infections, cirrhosis, hepatitis, and rejection of kidney transplants.

Molecular Genetics

The C3 gene is a 43 Kb stretch of DNA located on chromosome 19p. This gene, which consists of 41 exons. It codes for a C3 precursor (pro-C3), which weighs approximately 180KDa, and consists of 1663 amino acids. This pro-C3 protein has been shown to have significant sequence homology to alpha 2-microglobulin and C4. The processing of this C3 precursor involves the removal of four Arginine residues, producing two chains, of approximately 110 and 75 KDa, bound by a disulfide bond.

Epidemiology in the Arab World

View Map
Variant NameCountryGenomic LocationClinvar Clinical SignificanceCTGA Clinical Significance Condition(s)HGVS ExpressionsdbSNPClinvar
NM_000064.4:c.3343G>ASaudi ArabiaNC_000019.10:g.6692971C>TUncertain SignificancePathogenicHemolytic Uremic Syndrome, Atypical, Susceptibility To, 5NG_009557.1:g.32681G>A; NM_000064.4:c.3343G>A; NP_000055.2:p.Asp1115Asn12190958517062

Other Reports

Kuwait

See: Palestine > Pussell et al., 1980.

Lebanon

See: Palestine > Pussell et al., 1980.

Oman

White et al. (1997) measured the levels of C3 and C4 in 100 healthy Omani adults (50 males and 50 females) to obtain local reference values (which were compared to those of other populations) to be used when evaluating patients from the same population. The subjects were university students, blood donors, and healthy potential bone marrow and kidney donors and were not related to each other. Complement levels were measured by nephelometry, and the results were analyzed statistically. The mean of C3 levels was found to be 7.44 SD 1.86 micro-mol/L (min value of 4.8, max value of 13.6) with 95% confidence interval of 7.1- 7.8. These values did not differ between males and females, and they were not different from those of other Western populations.

Palestine

Pussell et al. (1980) described a family in which 3 children had homozygous C3 deficiency and both parents and 2 other children were heterozygous for a C3 null gene. A heterozygous child had membranoproliferative glomerulonephritis. Proteinuria and/or microscopic hematuria were present in all 3 homozygous children. The homozygous and heterozygous children were susceptible to infection. The only child with normal complement had neither nephritis nor increased susceptibility to infection. The family was of Palestinian-Lebanese origin, living in Kuwait. The parents were thought to be cousins.

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