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Complement component 4B

C4B, Complement C4b
This gene encodes the basic 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. 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. In addition, this gene exists as a long form and a short form due to the presence or absence of a 6.4 kb endogenous HERV-K retrovirus in intron 9. [provided by RefSeq, Jul 2008] (from NCBI)
Top mentioned proteins: C-4, HAD, MHC, 21-hydroxylase, DR3
Papers on C4B
A targeted proteomic strategy for the measurement of oral cancer candidate biomarkers in human saliva.
New
MacCoss et al., Campinas, Brazil. In Proteomics, Jan 2016
Using Skyline software package, we demonstrated a statistically significant higher abundance of the C1R, LCN2, SLPI, FAM49B, TAGLN2, CFB, C3, C4B, LRG1, SERPINA1 candidate biomarkers in the saliva of OSCC patients.
Gene copy-number variations (CNVs) of complement C4 and C4A deficiency in genetic risk and pathogenesis of juvenile dermatomyositis.
New
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.
New
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).
Genomic Copy Number Variations of the Complement Component C4B Gene Are Associated With Chronic Central Serous Chorioretinopathy.
New
de Jong et al., Nijmegen, Netherlands. In Invest Ophthalmol Vis Sci, Aug 2015
METHODS: C4A and C4B copy numbers were analyzed in 197 cCSC patients and 303 healthy controls by using a Taqman copy number determination assay.
Production of Monoclonal Antibodies in Serum-free Media.
New
Di Ventura et al., Teramo, Italy. In Monoclon Antib Immunodiagn Immunother, Aug 2015
Four hybridoma cell lines (C4B, 10C2G5, 6C5F4C7, 2D10G11) were adapted to grow in serum-free conditions.
Complement system in dermatological diseases - fire under the skin.
Review
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.
Genetics of leprosy reactions: an overview.
Review
Schurr et al., Montréal, Canada. In Mem Inst Oswaldo Cruz, 2012
Indeed, polymorphisms in seven genes [Toll-like receptors (TLR)1, TLR2, nucleotide-binding oligomerisation domain containing 2, vitamin D receptor, natural resistance-associated macrophage protein 1, C4B and interleukin-6] have been found to be associated with one or more LR outcomes.
Increased frequency of complement C4B deficiency in rheumatoid arthritis.
GeneRIF
Yu et al., United States. In Arthritis Rheum, 2012
Our findings indicate a relationship between C4B copy number variation and rheumatoid arthritis.
Analysis of binding sites on complement factor I using artificial N-linked glycosylation.
GeneRIF
Blom et al., Malmö, Sweden. In J Biol Chem, 2012
all analyzed cofactors form similar trimolecular complexes with FI and C3b/C4b, and the accessibility of FIMAC and SP domains is crucial for the function of FI
Genetically determined partial complement C4 deficiency states are not independent risk factors for SLE in UK and Spanish populations.
GeneRIF
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.
Inhibition of CXCL10 release by monomeric C3bi and C4b.
GeneRIF
Wakabayashi et al., Nishinomiya, Japan. In Clin Exp Immunol, 2012
Purified C4b inhibited IFN-beta-induced CXCL10 production & STAT1 phosphorylation.
[Differential expression and significance of complement C4b and transthyretin in proliferative vitreoretinopathy].
GeneRIF
Li et al., Wenzhou, China. In Zhonghua Yan Ke Za Zhi, 2011
There is a connection between elevated concentrations of both complement C4b and TTR and the pathogenesis of proliferative vitreoretinopathy.
Phenotypes, genotypes and disease susceptibility associated with gene copy number variations: complement C4 CNVs in European American healthy subjects and those with systemic lupus erythematosus.
Review
Yu et al., Columbus, United States. In Cytogenet Genome Res, 2007
A direct outcome of C4 CNV is the generation of two classes of polymorphic proteins, C4A and C4B, with differential chemical reactivities towards peptide or carbohydrate antigens, and a range of C4 plasma protein concentrations (from 15 to 70 mg/dl) among healthy subjects.
Diseases associated with the low copy number of the C4B gene encoding C4, the fourth component of complement.
Review
Fust et al., Budapest, Hungary. In Cytogenet Genome Res, 2007
In this review article, earlier and recent work of the research group on the copy number polymorphism of the C4B gene are summarized.
Diversity of the CYP21P-like gene in CYP21 deficiency.
Review
Lee, Shancheng, China. In Dna Cell Biol, 2005
The seven different CYP21 alleles produced 3.2 kb Taq I fragments caused by deletion of the RP2-XA-C4B locus.
The reaction mechanism of the internal thioester in the human complement component C4.
Impact
Law et al., Oxford, United Kingdom. In Nature, 1996
A two-step mechanism is used by the C4B isotype.
Rare variant of complement C4 is seen in high frequency in patients with primary glomerulonephritis.
Impact
Feucht et al., In Lancet, 1984
59 unselected patients with primary glomerulonephritis were phenotyped for alleles of the MHC-linked complement genes, C4A, C4B, and BF.
The structural basis of the multiple forms of human complement component C4.
Impact
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.
Impact
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.
A molecular basis for the two locus model of human complement component C4.
Impact
Rittner et al., In Nature, 1982
O'Neill et al. showed that this C4 polymorphism was controlled by two closely linked genetic loci, F (C4A) and S (C4B) and these results were extended by Awdeh et al. with an improved typing method.
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