| 產(chǎn)品編號 | bs-0096R-BF594 |
| 英文名稱(chēng) | Rabbit Anti-alpha 1 Antitrypsin/BF594 Conjugated antibody |
| 中文名稱(chēng) | BF594標記的α-1抗胰蛋白酶抗體 |
| 別 名 | A1-Antitrypsin; Tryptase; Alpha-1-Antitrypsin; alpha 1-antitrypsin; A1A; A1AT; AAT; Alpha 1 antiproteinase; Alpha 1 protease inhibitor; alpha-1-AT; alpha1 PI; alpha1 proteinase inhibitor; Clade A (alpha 1 antiproteinase antitrypsin) member 1; MGC23330; MGC9222; PI; PI; PRO2275; Protease inhibitor 1 (anti elastase); Serpin A1; Serpin peptidase inhibitor clade A (alpha 1 antiproteinase antitrypsin) member 1; SerpinA1; A1AT_HUMAN; Alpha-1-antitrypsin; Alpha-1 protease inhibitor; Alpha-1-antiproteinase; Serpin A1. |
| 規格價(jià)格 | 100ul/2980元 購買(mǎi) 大包裝/詢(xún)價(jià) |
| 說(shuō) 明 書(shū) | 100ul |
| 研究領(lǐng)域 | 心血管 免疫學(xué) 新陳代謝 |
| 抗體來(lái)源 | Rabbit |
| 克隆類(lèi)型 | Polyclonal |
| 交叉反應 | Human, Rat, (predicted: Mouse, Dog, Pig, Cow, ) |
| 產(chǎn)品應用 | ICC=1:50-200 IF=1:50-200
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 44kDa |
| 性 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human alpha 1 Antitrypsin |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 儲 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存條件 | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
| 產(chǎn)品介紹 |
background: Alpha-1-antitrypsin is a protease inhibitor, deficiency of which is associated with emphysema and liver disease. The protein is encoded by a gene (PI) located on the distal long arm of chromosome 14. Function: 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. Short peptide from AAT (SPAAT) is a 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). Subcellular Location: Secreted. Short peptide from AAT: Secreted, extracellular space, extracellular matrix. Tissue Specificity: Plasma. Post-translational modifications: N-glycosylated. Differential glycosylation produces a number of isoforms. N-linked glycan at Asn-107 is alternatively di-antennary, tri-antennary or tetra-antennary. The glycan at Asn-70 is di-antennary with trace amounts of tri-antennary. Glycan at Asn-271 is exclusively di-antennary. Structure of glycans at Asn-70 and Asn-271 is Hex5HexNAc4. The structure of the antennae is Neu5Ac(alpha1-6)Gal(beta1-4)GlcNAc attached to the core structure Man(alpha1-6)[Man(alpha1-3)]Man(beta1-4)GlcNAc(beta1-4)GlcNAc. Some antennae are fucosylated, which forms a Lewis-X determinant. Proteolytic processing may yield the truncated form that ranges from Asp-30 to Lys-418. DISEASE: Defects in SERPINA1 are the cause of alpha-1-antitrypsin deficiency (A1ATD) [MIM:613490]. A disorder whose most common manifestation is emphysema, which becomes evident by the third to fourth decade. A less common manifestation of the deficiency is liver disease, which occurs in children and adults, and may result in cirrhosis and liver failure. Environmental factors, particularly cigarette smoking, greatly increase the risk of emphysema at an earlier age. Similarity: Belongs to the serpin family. Database links: Entrez Gene: 5265 Human Omim: 107400 Human SwissProt: P01009 Human SwissProt: P07758 Mouse Unigene: 525557 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. α1-抗胰蛋白酶（A1AT）缺乏是最常見(jiàn)的遺傳代謝病，能引起肺和肝的損傷。α1抗胰蛋白酶為呼吸系統的非特異性可溶因子，與呼吸道抵抗力關(guān)系密切，它可抑制多種酶的活性，包括細菌的酶，以及中性白細胞溶酶體分泌的蛋白酶、彈性蛋白酶、膠原酶、纖維蛋白溶酶和凝血酶。α1抗胰蛋白酶的缺乏與慢性阻塞性肺病的形成關(guān)系密切，因為它的缺乏，不能及時(shí)控制感染和炎癥產(chǎn)生的多種蛋白酶，而造成肺組織破壞。 α1-抗胰蛋白酶由肝細胞產(chǎn)生,是一種分子量為45-56kDa的糖蛋白,它能抑制蛋白酶、彈性蛋白酶、膠原酶等多種水解酶的活性。 AAT也是一種糖蛋白，主要用于遺傳性AAT缺乏癥和良性惡性肝腫瘤、內胚竇瘤、組織細胞性淋巴瘤以及胰腺癌、胃癌、結腸癌等各種腫瘤的研究。此抗體與大、小鼠、狗和豬有交叉反應。 |
