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Complement component 4A

C-4, C4A, Complement C4a, Complement C4b, S-S, Complement C4
This gene encodes the acidic form of complement factor 4, part of the classical activation pathway. The protein is expressed as a single chain precursor which is proteolytically cleaved into a trimer of alpha, beta, and gamma chains prior to secretion. The trimer provides a surface for interaction between the antigen-antibody complex and other complement components. The alpha chain may be cleaved to release C4 anaphylatoxin, a mediator of local inflammation. Deficiency of this protein is associated with systemic lupus erythematosus and type I diabetes mellitus. This gene localizes to the major histocompatibility complex (MHC) class III region on chromosome 6. Varying haplotypes of this gene cluster exist, such that individuals may have 1, 2, or 3 copies of this gene. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Nov 2011] (from NCBI)
Top mentioned proteins: C4B, HAD, MHC, DR3, CAN
Papers on C-4
Gene copy-number variations (CNVs) of complement C4 and C4A deficiency in genetic risk and pathogenesis of juvenile dermatomyositis.
Yu et al., Columbus, United States. In Ann Rheum Dis, Nov 2015
We assess gene copy-number variations (CNVs) for complement C4 and its isotypes, C4A and C4B, in genetic risks and pathogenesis of JDM.
Novel Associations Between Major Histocompatibility Complex and Pediatric-Onset Inflammatory Bowel Disease.
Lokki et al., Helsinki, Finland. In J Pediatr Gastroenterol Nutr, Oct 2015
HLA-A, -B, -DRB1 alleles and complement C4A and C4B gene copy numbers were determined and constructed into haplotypes by a Bayesian algorithm (PHASE).
Comparative study of serum proteomes in Legg-Calve-Perthes disease.
Bai et al., Xi'an, China. In Bmc Musculoskelet Disord, 2014
Including higher abundances of complement factor H (1.44), complement C4-B (1.45), isocitrate dehydrogenase [NAD] subunit alpha (2.7) alpha-1-acid glycoprotein 1 (1.87), heptoglobin (1.53) and Ig lambda-2 chain C regions (1.46), and lower levels of apolipoprotein E (0.50), apolipoprotein F (0.60), apolipoprotein C-III (0.69), S100-A8 (0.73), S100-A9 (0.75) and prothrombin (0.77) in LCPD than in controls.
Do follicular dendritic cells regulate lupus-specific B cells?
Carroll et al., Boston, United States. In Mol Immunol, 2014
In this review we describe recently published results in which a B cell receptor-knock-in mouse strain specific for nucleolar self-antigens was bred with mice deficient in complement C4 and discuss the implications for the lupus field.
Complement system in dermatological diseases - fire under the skin.
Meri et al., Helsinki, Finland. In Front Med (lausanne), 2014
On one hand, drugs with free hydroxyl on amino groups (e.g., hydralazine, procainamide) could interact with C4A, C4B, or C3 and cause an SLE-like disease.
Complement C4 induces regulatory T cells differentiation through dendritic cell in systemic lupus erythematosus.
Shi et al., Chengdu, China. In Cell Biosci, 2014
Complement component 4 (C4) has be proved to play a role in pathogenesis of SLE.
Proteomic identification of alpha-2-HS-glycoprotein as a plasma biomarker of hypopharyngeal squamous cell carcinoma.
Li et al., Guangzhou, China. In Int J Clin Exp Pathol, 2014
The up-regulation proteins were alpha-2-HS-glycoprotein (AHSG), complement C4-B, haptoglobin, C-reactive protein, and ceruloplasmin, whereas the down-regulation proteins were serum albumin, angiotensinogen, alpha-1-antichymotrypsin, Ig gamma-3 chain C region, fibrinogen gamma chain, apolipoprotein A-I, and Ig kappa chain C region.
Immunogenetic factors affecting susceptibility of humans and rodents to hantaviruses and the clinical course of hantaviral disease in humans.
Vaheri et al., Montpellier, France. In Viruses, 2014
The polymorphism of other immunity-related genes including the C4A gene and a high-producing genotype of TNF gene associated with severe PUUV infection.
Proteomic biomarkers of endometrial cancer risk in women with polycystic ovary syndrome: a systematic review and biomarker database integration.
Atiomo et al., Nottingham, United Kingdom. In Gynecol Endocrinol, 2013
These include transgelin, pyruvate kinase M1/M2, gelsolin-like capping protein (macrophage capping protein), glutathione S-transferase P, leucine aminopeptidase (cytosol aminopeptidase), peptidyl-prolyl cis-transisomerase, cyclophilin A, complement component C4A and manganese-superoxide dismutase.
Copy number variation in autoimmunity--importance hidden in complexity?
Holmdahl et al., Stockholm, Sweden. In Eur J Immunol, 2012
Nonetheless, CNVs in FCGR3B, DEFB4, CCL3L1, C4A/B and NCF1 have been suggested to be associated with autoimmune diseases, although there is conflicting evidence in all cases.
Functional annotation of the human fat cell secretome.
Arner et al., Huddinge, Sweden. In Arch Physiol Biochem, 2012
Complement C4a gene expression is regulated both by obesity and by the region between visceral and subcutaneous adipose tissue.
C4.4A is associated with tumor budding and epithelial-mesenchymal transition of colorectal cancer.
Mori et al., Ōsaka, Japan. In Cancer Sci, 2012
findings suggest that a tight association between C4.4A and tumor budding may, in part, be due to C4.4A promoting epithelial-mesenchymal transition at the invasive front of colorectal cancer
Pros and cons for C4d as a biomarker.
Bajema et al., Leiden, Netherlands. In Kidney Int, 2012
Past, present, and future perspectives of C4d as a biomarker, focusing on its use in solid organ transplantation and discussing its possible new roles in autoimmunity and pregnancy. Review.
Genetically determined partial complement C4 deficiency states are not independent risk factors for SLE in UK and Spanish populations.
Fernando et al., London, United Kingdom. In Am J Hum Genet, 2012
Although complete homozygous deficiency of complement C4 is one of the strongest genetic risk factors for SLE, partial C4 deficiency states do not independently predispose to the disease.
Membrane-bound and exosomal metastasis-associated C4.4A promotes migration by associating with the α(6)β(4) integrin and MT1-MMP.
Zöller et al., Heidelberg, Germany. In Neoplasia, 2012
the first explanation for the C4.4A contribution to wound healing and metastasis.
Capillary leak syndrome in children with C4A-deficiency undergoing cardiac surgery with cardiopulmonary bypass: a double-blind, randomised controlled study.
Hu et al., Wuhan, China. In Lancet, 2005
On the basis of previous results, we undertook a randomised controlled study to investigate the priming with plasma rich in the C4A isotype of complement component 4 on the incidence of capillary leak syndrome in children with C4A deficiency.
Hereditary angioedema with normal C1-inhibitor activity in women.
Traupe et al., Mainz, Germany. In Lancet, 2000
We measured C1-inhibitor concentration and functional activity as well as complement C4 concentration and took pedigrees to characterise patients.
The reaction mechanism of the internal thioester in the human complement component C4.
Law et al., Oxford, United Kingdom. In Nature, 1996
A key step in the elimination of pathogens from the body is the covalent binding of complement proteins C3 and C4 to their surfaces.
The structural basis of the multiple forms of human complement component C4.
Porter et al., In Cell, 1984
cDNA clones of human complement components C4A and C4B alleles were prepared from mRNA obtained from the liver of a donor heterozygous at both loci.
Correlation between a DNA restriction fragment length polymorphism and C4A6 protein.
Carroll et al., In Nature, 1984
The fourth component of complement (C4) in man, is coded for by two separate but closely linked loci (C4A and C4B) within the major histocompatibility region (MHC), on the short arm of chromosome 6.
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