SERPINA1 Recombinant Monoclonal Antibody

說明書

中文名稱：

SERPINA1 Recombinant Monoclonal Antibody
貨號：

CSB-RA977406A0HU
規格：

￥1320
圖片：
Western Blot
Positive WB detected in:HEK293 whole cell lysate(30μg), Hela whole cell lysate(30μg), Jurkat whole cell lysate(30μg), Mouse liver tissue lysate(30μg)
All lanes:Antithrombin 3 antibody at 1:1000
Secondary
Goat polyclonal to rabbit IgG at 1/40000 dilution
Observed band size: 47 kDa
Exposure time：1min

Immunofluorescence staining of HepG2 cell with CSB-RA977406A0HU at 1:50, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).

Overlay Peak curve showing HepG2 cells stained with CSB-RA977406A0HU (red line) at 1:100. The cells were fixed in 4% formaldehyde and permeated by 0.2% TritonX-100 for 10min. Then 10% normal goat serum to block non-specific protein-protein interactions followed by the antibody (1ug/1*10⁶cells) for 45min at 4℃. The secondary antibody used was FITC-conjugated goat anti-rabbit IgG (H+L) at 1/200 dilution for 35min at 4℃.Control antibody (green line) was Rabbit IgG (1ug/1*10⁶cells) used under the same conditions. Acquisition of >10,000 events was performed.
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

Uniprot No.：

P01009
基因名：

SERPINA1
別名：

Alpha-1-antitrypsin (Alpha-1 protease inhibitor) (Alpha-1-antiproteinase) (Serpin A1) [Cleaved into: Short peptide from AAT (SPAAT)], SERPINA1, AAT PI
反應種屬：

Human, Mouse
免疫原：

A synthesized peptide from human SERPINA1 protein
免疫原種屬：

Homo sapiens (Human)
標記方式：

Non-conjugated
克隆類型：

Monoclonal
抗體亞型：

Rabbit IgG
純化方式：

Affinity-chromatography
克隆號：

11G10
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
應用范圍：

ELISA, WB, IF, FC

推薦稀釋比：

Application	Recommended Dilution
WB	1:500-1:2000
IF	1:50-1:200
FC	1:50-1:200

Protocols：

ELISA Protocol
Western Blotting (WB) Protocol
Immunofluorescence (IF) Protocol
Flow cytometry (FC) Protocol
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：

Inhibitor of serine proteases. Its primary target is elastase, but it also has a moderate affinity for plasmin and thrombin. Irreversibly inhibits trypsin, chymotrypsin and plasminogen activator. The aberrant form inhibits insulin-induced NO synthesis in platelets, decreases coagulation time and has proteolytic activity against insulin and plasmin.; reversible chymotrypsin inhibitor. It also inhibits elastase, but not trypsin. Its major physiological function is the protection of the lower respiratory tract against proteolytic destruction by human leukocyte elastase (HLE).
基因功能參考文獻：
1. lower levels of serum alpha-1 antitrypsin were more frequent in nonidiopathic interstitial pneumonia patients than idiopathic interstitial pneumonia without an increase in genetic polymorphism; the difference was not statistically significant PMID: 28425234
2. High A1AT expression is associated with HIV infection. PMID: 29351445
3. Mutation GLU342LYS (rs28929474) and GLU264VAL (rsl7S80) in gene SERPINAl in patients with various stages of Pneumoconiosis correlates with early development and unfavorable course of Pneumoconiosis in post-contact period. PMID: 30351692
4. Mutations in the alpha-1-antitrypsin and Wilson's genes may act as cofactors in the pathogenesis of fatty liver diseases. PMID: 29324588
5. Data suggest alpha-1-antitrypsin (A1AT) and APOH (beta-2-glycoprotein-1) could represent promising markers for renal carcinogenesis in Von Hippel-Lindau disease (VHLD) patients. PMID: 28004973
6. The two genetic associations with severe liver disease that had been suspected previously (one SNP for SERPINA1 and another for MAN1B1) were not confirmed in our cohort. Moreover, the haplotype analysis identified only one major genetic background for the SERPINA1 Z-allele, allowing us to exclude the presence of a frequent modifier SNP within. PMID: 28887821
7. The reduction in circulating AAT levels associated to the PI*Zla palma allele was similar to that of PI*Z allele, representing a risk of impairment in lung function. PMID: 28947017
8. Undifferentiated ASC may require priming to secrete functional A1AT, a potent antiprotease that may be relevant to stem cell therapeutic effects. PMID: 28825379
9. The high expression of SERPINA1 indicates a poor prognosis of high-grade gliomas PMID: 29309973
10. associations of PiMZ genotype with chronic obstructive pulmonary disease, lung function, and emphysema in whites and African Americans PMID: 28380308
11. A1AT polymorphisms may be associated with human retrovirus infections in an ethnically mixed population from the Amazon region of Brazil. PMID: 29030134
12. Results provide evidence that a non-synonymous single-nucleotide variant in SERPINA1 gene encoding alpha-1 antitrypsin as a major risk gene for large artery stroke. PMID: 28265093
13. Compound heterozygous Alpha-1 antitrypsin Pi*SZ genotype is relatively frequent in Caucasian subjects resident in Europe, and in their descendants residing in the American continent, Australia, and New Zealand. PMID: 28652721
14. The mean cerebrospinal fluid signals of the most acidic serpinA1 isoform differed significantly between Parkinson patients with dementia, without dementia and healthy controls. Patients above the cut-off of 6.4 have a more than six times higher risk for an association with dementia compared to patients below the cut off. PMID: 27184740
15. This study suggested that high a1-antitrypsin (AAT)expression might be a negative prognostic marker for lung adenocarcinoma. AAT promoted lung adenocarcinoma metastasis, whose functional target may be fibronectin . Our findings provide new insight into the mechanisms of lung adenocarcinoma metastasis PMID: 28440399
16. this study identified a carbamylated peptide of A1AT as an antigenic target of autoantibodies in patients with rheumatoid arthritis PMID: 28291659
17. Novel RNA-binding activity of NQO1 promotes SERPINA1 mRNA translation. PMID: 27515817
18. ZAAT ER clearance resulted from enhancing ERdj3-mediated ZAAT degradation by silencing ERdj3 while simultaneously enhancing autophagy. In this context, ERdj3 suppression may eliminate the toxic gain of function associated with polymerization of ZAAT PMID: 28419579
19. In this study, the contribution of rare SERPINA1 alleles into AATD should not be neglected in the diagnosis practice given there is a wide spectrum of variants originated by mutation and sometimes shuffled between chromosomes by recombination. PMID: 27296815
20. evaluation of serum proteins reveals that SERPINA1, SERPINA3 and SERPINC1 could be useful to discriminate healthy from colorectal carcinoma patients with a high sensitivity and specificity PMID: 27492143
21. Alpha-1-antitrypsin for the treatment of steroid-refractory acute gastrointestinal graft-versus-host disease. PMID: 28699171
22. this study proved that SYVN1 enhances SERPINA1(E342K)/ATZ degradation through SQSTM1-dependent autophagy and attenuates SERPINA1(E342K)/ATZ cytotoxicity. PMID: 28121484
23. Recombinant human alpha-1 antitrypsin-Fc fusion protein reduces acute myocardial inflammatory injury after ischemia-reperfusion in the mouse leading to preservation of viable myocardium and systolic function. PMID: 26945157
24. report association of APOE and TOMM40 with behavioural variant frontotemporal dementia, and ARHGAP35 and SERPINA1 with progressive non-fluent aphasia. PMID: 28387812
25. Our results indicated a high prevalence of SERPINA1 alleles in the normal Saudi population and an association between alpha-1 antitrypsin deficiency and COPD development. PMID: 29049242
26. findings highlight A1AT as a potential serum scavenger of hemin and suggest that the commercial preparations of human plasma A1AT might prove to be useful therapeutics in conditions associated with hemolysis. PMID: 28716864
27. Reduced alpha-1-antitrypsin and elevated IL-23 csf levels were seen in amyotrophic lateral sclerosis patients. PMID: 27245439
28. the results of this study demonstrate a previously unknown role for P2 residue in determining inhibitory specificity of A1AT. PMID: 28922398
29. high A1AT expression correlated with increased tumor stage, elevated p53 staining and shorter overall survival in lung adenocarcinoma patients. PMID: 28368395
30. These results clearly demonstrate that the association of malectin with ribophorin I is required to capture misfolded alpha1-antitrypsin and direct them to the degradation pathway. PMID: 25451265
31. nNIF peptide is an inhibitor of NET formation in umbilical cord blood. PMID: 27599294
32. AAT heterozygous status in rheumatoid arthritis is strongly associated with positive anti-citrullinated peptide autoantibodies and may define a distinct subset of patients with increased disease severity. PMID: 28409899
33. Report alpha-1 antitrypsin Pi*Z gene frequency and Pi*ZZ genotype numbers worldwide. PMID: 28243076
34. SNP rs8004738 of SERPINA1 gene significantly interacted with smoking status and was associated with a higher risk for COPD in the Chinese population. PMID: 28138235
35. tissue specific expression and regulation of short transcripts of SERPINA1 gene and the presence of C-terminal peptides in supernatants from activated neutrophils PMID: 28107454
36. SERPINA1 variants are associated with ANCA-associated vasculitis risk. PMID: 28029757
37. Although the observed effect size of hAAT administration was limited and therefore the clinical relevance of our findings should be evaluated carefully, these data support the potential of this natural protein to ameliorate ischemic and inflammatory conditions. PMID: 28235038
38. Report incorporation of the Mmalton allele-specific genotyping assay in the diagnostic algorithm of alpha-1-antitrypsin deficiency. PMID: 27877030
39. The expression and intracellular accumulation of mutated Z-alpha-1 antitrypsin (the most common mutation) compromises macrophage function and contributes to emphysema development. Macrophages from individuals with Z-AAT have more proteolytic activity on ECM than normal. This abnormal activity is not abrogated by exogenous AAT and may be the result of cellular dysfunction induced by intracellular accumulation of Z-AAT. PMID: 28362108
40. hAAT inhibits DC activation and function, and it also attenuates autoimmunity and renal damage in the MRL/lpr lupus model PMID: 27232337
41. The deficiency PI*Z A1AT allele is considerably more common in patients with chronic respiratory disorders than in the general Polish population. PMID: 26987331
42. Norursodeoxycholic acid administration is associated with increased autophagy, reduced A1AT protein accumulation, and reduced liver injury in a model of A1AT deficiency. PMID: 27102560
43. Fecal enzyme-linked immunosorbent assay showed that alpha-1-antitrypsin level was able to distinguish cholangiocarcinoma patients from normal individuals. In conclusion, alpha-1-antitrypsin is a potential marker for early diagnosis of cholangiocarcinoma PMID: 28618946
44. Reactive center loop insertion in alpha 1-Antitrypsin captured by accelerated molecular dynamics simulation has been presented. PMID: 27995800
45. This aberrant conformation, induced by the loss of interactions from the Glu-342 side chain, explains why Z alpha1-AT is prone to polymerization and readily binds to a 6-mer peptide, and it supports that annealing of s5A into the central beta-sheet is a crucial step in the serpins' metastable conformational formation. The demonstration that the aberrant conformation can be rectified through stabilization of the labile ... PMID: 27246852
46. The Alpha-1 antitrypsin (A1AT) deficiency (A1ATD) is a genetic autosomal recessive disease, characterized by low serum levels of A1AT. PMID: 27855621
47. This work demonstrates a simple and efficient one-step purification of ralpha1-PI from transgenic plants, which is an essential prerequisite for further therapeutic development. PMID: 26852026
48. Altered glycosylation, expression of serum haptoglobin and alpha-1-antitrypsin in chronic hepatitis C, hepatitis C induced liver cirrhosis and hepatocellular carcinoma patients. PMID: 27034286
49. The PI-MZ rare (2%) SNP effect is nearly four times greater than the 'top' common height SNP in HMGA2. However, height only partially attenuates the SERPINA1-FEV1 or FVC association (around 50%) and vice versa PMID: 26831755
50. We aimed to explore the features of Chronic Obstructive Pulmonary Disease in subjects carrying the PIMMmalton mutation, which is the most prevalent alpha-1 antitrypsin variant in Tunisia PMID: 26071129
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相關疾病：

Alpha-1-antitrypsin deficiency (A1ATD)
亞細胞定位：

Secreted. Endoplasmic reticulum. Note=The S and Z allele are not secreted effectively and accumulate intracellularly in the endoplasmic reticulum.; [Short peptide from AAT]: Secreted, extracellular space, extracellular matrix.
蛋白家族：

Serpin family
組織特異性：

Ubiquitous. Expressed in leukocytes and plasma.
數據庫鏈接：

HGNC: 8941

OMIM: 107400

KEGG: hsa:5265

STRING: 9606.ENSP00000348068

UniGene: Hs.525557

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