C4A Antibody, FITC conjugated

說明書

中文名稱：

C4A兔多克隆抗體, FITC偶聯
貨號：

CSB-PA003949LC01HU
規格：

￥880
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) C4A Polyclonal antibody
Uniprot No.：

P0C0L4
基因名：

C4A
別名：

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
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human Complement C4-A protein (1027-1186AA)
免疫原種屬：

Homo sapiens (Human)
標記方式：

FITC
克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

>95%, Protein G purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
產品提供形式：

Liquid
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期：

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.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

功能：

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.
基因功能參考文獻：
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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相關疾病：

Complement component 4A deficiency (C4AD); Systemic lupus erythematosus (SLE)
亞細胞定位：

Secreted. Cell junction, synapse. Cell projection, axon. Cell projection, dendrite.
組織特異性：

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
數據庫鏈接：

HGNC: 1323

OMIM: 120790

KEGG: hsa:720

STRING: 9606.ENSP00000396688

UniGene: Hs.534847

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