C4A Antibody, FITC conjugated

Code CSB-PA003949LC01HU
Size US$166
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Product Details

Full Product Name
Rabbit anti-Homo sapiens (Human) C4A Polyclonal antibody
Uniprot No.
Target Names
C4A
Alternative Names
Acidic C4 antibody; Acidic complement C4 antibody; Basic C4 antibody; Basic complement C4 antibody; C3 and PZP-like alpha-2-macroglobulin domain-containing protein 2 antibody; C3 and PZP-like alpha-2-macroglobulin domain-containing protein 3 antibody; C4; Chido form antibody; C4; Rodgers from antibody; C4A anaphylatoxin antibody; C4A antibody; C4A2 antibody; C4A3 antibody; C4A4 antibody; C4A6 antibody; C4AD antibody; C4B antibody; C4B_2 antibody; C4B1 antibody; C4B12 antibody; C4B2 antibody; C4B3 antibody; C4BD antibody; C4F antibody; C4S antibody; CH antibody; Chido form of C4 antibody; CO4 antibody; CO4A_HUMAN antibody; Complement C4 A antibody; Complement C4 B antibody; Complement C4 gamma chain antibody; complement C4-A antibody; complement C4-B antibody; complement C4-B-like antibody; complement C4B1a antibody; Complement component 4A (Rodgers blood group) antibody; Complement component 4A antibody; complement component 4B (Chido blood group) antibody; complement component 4B (Chido blood group); copy 2 antibody; Complement component 4B (Childo blood group) antibody; Complement component 4B antibody; Complement component 4F antibody; Complement component 4S antibody; CPAMD2 antibody; CPAMD3 antibody; RG antibody; Rodgers form of C4 antibody
Raised in
Rabbit
Species Reactivity
Human
Immunogen
Recombinant Human Complement C4-A protein (1027-1186AA)
Immunogen Species
Homo sapiens (Human)
Conjugate
FITC
Clonality
Polyclonal
Isotype
IgG
Purification Method
>95%, Protein G purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Form
Liquid
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

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Target Background

Function
Non-enzymatic component of C3 and C5 convertases and thus essential for the propagation of the classical complement pathway. Covalently binds to immunoglobulins and immune complexes and enhances the solubilization of immune aggregates and the clearance of IC through CR1 on erythrocytes. C4A isotype is responsible for effective binding to form amide bonds with immune aggregates or protein antigens, while C4B isotype catalyzes the transacylation of the thioester carbonyl group to form ester bonds with carbohydrate antigens.; Derived from proteolytic degradation of complement C4, C4a anaphylatoxin is a mediator of local inflammatory process. It induces the contraction of smooth muscle, increases vascular permeability and causes histamine release from mast cells and basophilic leukocytes.
Gene References into Functions
  1. The purpose of this study was to evaluate C4A and C4B in patients with congenital adrenal hyperplasia in relation to CYP21A2 genotype and psychiatric and autoimmune comorbidity. We determined the copy numbers of C4A and C4B in 145 patients with CAH .No association was found between C4 copy number and autoimmune disease. PMID: 30465166
  2. Low C4 in systemic lupus erythematosus patients is due to consumption rather than deficient synthesis related to lower C4A & B gene copy numbers. PMID: 30041577
  3. Report strong association of systemic lupus erythematosus in individuals with low copy numbers of C4 and in particular in patients with complete deficiency of C4A. PMID: 29050534
  4. An increase in serum C4, as wall as a decrease in C3, was an important outcome determinant for patients with immunoglobulin A nephropathy. PMID: 28697742
  5. for the first time, a complete overview of C4 in SLE from genetic variation to binding capacity using a novel test. As this test detects crossing over of Rodgers and Chido antigens, it will allow for more accurate measurement of C4 in future studies. PMID: 29080553
  6. The study re-evaluates low-resolution crystal structures of C4 via interactive molecular-dynamics flexible fitting. In terms of biology, the results provide a better structural framework for understanding the pivotal function of the C4 protein within the complement system. PMID: 27599733
  7. An elevated number of C4 genes was observed in Alzheimer's disease (AD) patients as compared with healthy controls. The presence of high C4A and C4B copy numbers in AD patients could explain the increased C4 protein expression observed in AD patients, thus highlighting a possible role for C4A and C4B copy number variations in the risk of developing AD. PMID: 27758680
  8. This study shows that the C4c/C4 ratio seems to be a better diagnostic measure than total antigenic C4 alone. Our findings underline that screening with total antigenic C4 implies a risk of overlooking C1-INH-HAE patients. PMID: 28412283
  9. In comparison with C4-intact patients, C4-deficient patients had a different clinical/serologic lupus-like phenotype and lacked the lupus interferon signature. PMID: 27274010
  10. C4 copy number variations and deficiency of C4A both play an important role in the risk and manifestations of systemic lupus erythematosus in East Asian and European populations PMID: 26814708
  11. Complement C4A deficiency appears to be an important factor for the genetic risk and pathogenesis of juvenile dermatomyositis, particularly in patients with a DR3-positive background. PMID: 26493816
  12. Coronary atherosclerosis is distinguished by serum C4 complement up-regulation and ceruloplasmin down-regulation. PMID: 28091899
  13. find strong statistical significance for association of increased copy number of C4A (OR 0.81 (0.73; 0.89);P = 4.4 x 10(-5)), with the effect most pronounced in individuals over 78 years (OR 0.67 (0.55; 0.81)) and females PMID: 27090374
  14. Copy Number Variation Scan Identifies Complement Component C4 as Novel Susceptibility Gene for Crohn's Disease. PMID: 26595553
  15. genetic polymorphism is associated with acute graft versus host disease in unrelated hematopoietic stem cell transplantation PMID: 26602146
  16. important role of complement C4a in inhibiting the HBV DNA secretion in chronic hepatitis b PMID: 26119402
  17. C4A and C4B gene copy numbers are stronger risk factors for juvenile-onset than for adult-onset systemic lupus erythematosus. PMID: 26800705
  18. Complement C4A deficiency (gene copy number <=1) was identified as a risk factor in a case-control study of juvenile dermatomyositis, particularly when subjects concurrently carried the HLA-DRB1*0301 allele. PMID: 26493816
  19. Increased age, rs2857009 single nucleotide polymorphism of complement component C4 and hepatitis C virus genotype were associated with disease progression. PMID: 25573496
  20. Low C4 was associated with non-Hodgkin's lymphoma in primary Sjogren's syndrome. PMID: 26359802
  21. Schizophrenia risk from complex variation of complement component 4 PMID: 26814963
  22. C4 levels were significantly lower in Factor XII-hereditary angioedema than in idiopathic non-histaminergic acquired angioedema. PMID: 25744496
  23. our study indicates that Finnish NTM patients had significantly more often C4 deficiencies than the healthy control subjects. PMID: 24638111
  24. Complement components C3a and C4a, but not C5a, display antimicrobial activity against P. aeruginosa, E. coli, B. subtilis, and C. albicans. PMID: 17132627
  25. C4 is a novel cellular substrate of the HCV NS3/4A protease. PMID: 24349192
  26. Low C4 gene copy numbers are associated with superior graft survival in patients transplanted with a deceased donor kidney. PMID: 23715124
  27. serum C4a desArg is a potential biomarker for the severity of histological findings in patients with IgA nephropathy. PMID: 23708385
  28. Our findings indicate that a high copy number of C4A confers risk for Behcet disease by modulating the expression of C4A and enhancing IL-6 production. PMID: 23918728
  29. Studies indicate that initiation of lectin compleme pathway leads to activation of the serine proteases MASP-1 and MASP-2 resulting in deposition of C4 on the activator and assembly of the C3 convertase. PMID: 23911397
  30. C4d might be a biomarker for evaluating the risk for IUGR and disease control in patients with systemic lupus erythematosus and pregnancy-induced hypertension. PMID: 23530559
  31. Studies indicate beta-defensins (DEFB4, DEFB103, DEFB104), chemokine ligand 3 like 1 (CCL3L1), Fc gamma receptor 3B (FCGR3B), and complement component C4 (C4) for copy number variation in disease association. PMID: 22837109
  32. Complement 4a plasma protein was identified as increased in Alzheimer's disease PMID: 22052466
  33. Deletion variants of C4 were found to be associated with SLE in Korean women, but did not reach statistical significance. PMID: 23335107
  34. study concludes that the association of C4 gene copy with systemic lupus erythematosus(SLE)was replicated in Chinese Han population, which highlighted the importance of C4 in SLE pathogenesis of diverse populations PMID: 21904924
  35. The precise order and size of all C4 genes were determined in RCCX, a multiallelic copy number variation locus. PMID: 22785613
  36. C4A deficiency is one of the minor defects of the innate immunity that may predispose children and young adults to recurrent respiratory infections. PMID: 22406254
  37. congenital adrenal hyperplasia patients have increased C4 copy number variation, with mutation-specific associations that may be protective for autoimmune disease PMID: 22886582
  38. C4A appears to associate with the protection of residual beta-cell function in new-onset type 1 diabetes. PMID: 22151770
  39. analysis of gene copy number of complement C4A, C4B and C4A silencing mutation by real-time quantitative polymerase chain reaction PMID: 22737222
  40. analysis of the structural basis for activation of the complement system by component C4 cleavage PMID: 22949645
  41. Complement C4a gene expression is regulated both by obesity and by the region between visceral and subcutaneous adipose tissue. PMID: 22616691
  42. 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. PMID: 22297669
  43. We showed no evidence for a role of hs-CRP, C3 and C4 in the association between BMI and asthma symptoms in overweight children. PMID: 21801245
  44. 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. PMID: 22387014
  45. Individuals with 4, 2, and 2 copies of C4, C4A and C4B genes, especially those with A2B2 polymorphism may associate with the development of Graves' disease PMID: 21943165
  46. Data show that in the UK cohort, total C4 GCN ranged from 2 to 6, with copy numbers from 0 to 4 observed for both C4A and C4B, while in the Spanish cohort, C4A GCN from 0 to 6 and C4B GCN from 0 to 5. PMID: 21857912
  47. The study shows the complement component C4A in the plasmas of sePE women is lower than the severe, late-onset PE women, and the Apolipoprotein A-I level is higher in sePE women than slPE women. PMID: 21677994
  48. C4 mRNA levels of the two isoforms (C4A and C4B) were significantly reduced in hepatocytes transfected with RNA from HCV genotype 1a or 2a. PMID: 21345967
  49. The reduction in olfactory function in these hereditary angioedema cases seems to correlate with complement C4 and CH50 levels. PMID: 20649895
  50. not demonstrate that C4 gene copy number associates with transplant outcome PMID: 21164027

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Involvement in disease
Complement component 4A deficiency (C4AD); Systemic lupus erythematosus (SLE)
Subcellular Location
Secreted. Cell junction, synapse. Cell projection, axon. Cell projection, dendrite.
Tissue Specificity
Complement component C4 is expressed at highest levels in the liver, at moderate levels in the adrenal cortex, adrenal medulla, thyroid gland, and the kidney, and at lowest levels in the heart, ovary, small intestine, thymus, pancreas and spleen. The extr
Database Links

HGNC: 1323

OMIM: 120790

KEGG: hsa:720

STRING: 9606.ENSP00000396688

UniGene: Hs.534847

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