凝集素甘露糖結(jié)合蛋白1抗體,Anti-LMAN1抗體簡單介紹：

產(chǎn)品編號：BYK-18304R

英文名稱：Anti-LMAN1
中文名稱：凝集素甘露糖結(jié)合蛋白1抗體

別名：Endoplasmic reticulum golgi intermediate compartment protein 53; ER-Golgi intermediate compartment 53 kDa protein; ERGIC-53; ERGIC53; ERGIC53 like protein; F5F8D; FMFD1; Gp58; Intracellular mannose specific lectin; Intracellular mannose-specific lectin MR60; Lectin mannose binding 1; Lectin mannose-binding 1; Lman1; LMAN1 like protein; LMAN1_HUMAN; MCFD1; MR60; Protein ERGIC-53. 

背景介紹：The protein encoded by this gene is a type I integral membrane protein localized in the intermediate region between the endoplasmic reticulum and the Golgi, presumably recycling between the two compartments. The protein is a mannose-specific lectin and is a member of a novel family of plant lectin homologs in the secretory pathway of animal cells. Mutations in the gene are associated with a coagulation defect. Using positional cloning, the gene was identified as the disease gene leading to combined factor V-factor VIII deficiency, a rare, autosomal recessive disorder in which both coagulation factors V and VIII are diminished.

產(chǎn)品規(guī)格：50ul；100ul；200ul；1ml，*

抗體來源 Rabbit or Mouse

克隆類型 Polyclonal or Monoclonal

交叉反應(yīng)、產(chǎn)品應(yīng)用 （具體交叉反應(yīng)與產(chǎn)品應(yīng)用請或添加客服直接索?。?/span>

性    狀 Lyophilized or Liquid
濃    度 1mg/ml

凝集素甘露糖結(jié)合蛋白1抗體,Anti-LMAN1抗體產(chǎn)品信息：

免疫應(yīng)答

    抗原刺激機(jī)體產(chǎn)生相應(yīng)的抗體，抗體的產(chǎn)生過程即為體液免疫應(yīng)答過程。

    體液免疫應(yīng)答的主要*位是淋巴結(jié)、脾臟和粘膜下淋巴組織等外周免疫器官，外來抗原進(jìn)入機(jī)體后即被補(bǔ)體致敏或者被DC吞噬，然后隨淋巴液進(jìn)入引流淋巴結(jié)。淋巴濾泡中的FDC通過其表面的補(bǔ)體受體捕獲被致敏的外來抗原，激活B細(xì)胞。位于脾髓質(zhì)交界區(qū)的DC則為T細(xì)胞呈遞外來抗原肽，啟動獲得性免疫應(yīng)答，B細(xì)胞在外周成熟、發(fā)育過程的zui后階段是成為專門的合成和分泌抗體分子的漿細(xì)胞。漿細(xì)胞高爾基體發(fā)達(dá)、代謝活躍，但沒有繼續(xù)分裂、增殖的能力，也不參與抗原遞呈。他們寄居在脾臟或者骨髓中，集中所有的能量生產(chǎn)并分泌抗體分子。抗體分子的重鏈和輕鏈在內(nèi)質(zhì)網(wǎng)上合成、組裝后經(jīng)高爾基體運輸至細(xì)胞表面并分泌于胞外。一個漿細(xì)胞每秒鐘能分泌5000個抗體分子，在外周的漿細(xì)胞壽命僅為幾天，但是骨髓中的漿細(xì)胞壽命可能較長。

免疫應(yīng)答過程：

1.抗原進(jìn)入機(jī)體；

2.抗原刺激 B淋巴細(xì)胞，使之分化增殖成為抗體分泌漿細(xì)胞和記憶細(xì)胞（初次免疫應(yīng)答）；

3.抗體主要由漿細(xì)胞分泌 ；

4.記憶細(xì)胞再次受到特異性抗原刺激后會被激活而成為分泌抗體的漿細(xì)胞（再次免疫應(yīng)答）；

5.記憶B細(xì)胞是長命細(xì)胞，可以存活數(shù)月甚至機(jī)體終生

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