α-1抗胰蛋白酶抗體

規(guī)格：1mg/1ml

英文名： alpha 1 Antitrypsin

別名： A1-Antitrypsin; Tryptase; Alpha-1-Antitrypsin; alpha 1 Antitrypsin; A1A; A1AT; AAT; Alpha 1 antiproteinase; Alpha 1 antitrypsin; Alpha 1 protease inhibitor; alpha-1-AT; alpha1 PI; alpha1 proteinase in

分子量： 44kDa

儲存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce

克隆類型：Polyclonal

亞型：IgG

純化方法：affinity purified by Protein A

**原：KLH conjugated synthetic peptide derived from human alpha 1

交叉反應(yīng)：Human, Mouse, Rat, Dog, Pig, Cow,

α-1抗胰蛋白酶抗體細(xì)胞定位：細(xì)胞外基質(zhì) 分泌型蛋白

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: α-1抗胰蛋白酶抗體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 *****s, 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.α-1抗胰蛋白酶抗體 Gene ID: 5265 Database links: Entrez Gene: 5265 Human Entrez Gene: 24648 Rat Omim: 107400 Human SwissProt: P01009 Human SwissProt: P07758 Mouse SwissProt: P17475 Rat 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*常見的遺傳代謝病，能引起肺和肝的損傷。α1抗胰蛋白酶為呼吸系統(tǒng)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**的酶，以及中性白細(xì)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(xì)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(nèi)胚竇瘤、組織細(xì)胞性**瘤以及胰腺癌、胃癌、結(jié)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(yīng)。

α-1抗胰蛋白酶抗體產(chǎn)品應(yīng)用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蠟切片需做抗原修復(fù)） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.

研究領(lǐng)域：心血管  **學(xué)  新陳代謝  

儲存條件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

來源： Rabbit

外觀： Lyophilized or Liquid

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