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產品分類 > 與ANCA相關 > Proteinase 3 (PR3)/蛋白酶3

Proteinase 3 (PR3)/蛋白酶3

產品貨號:18600/18601
促銷: 0.00

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0.1mg 1mg
德國Diarect

Proteinase 3 (PR3)

Diseases:
Wegeners Granulomatosis
 

Proteinase 3 (PR3) is a neutral serine protease stored in the azurophilic neutrophilic granulocytes, consisting of 228 amino acids (MW 29-33 kDa depending on glycoform). It has substantial sequence homology with neutrophil elastase, cathepsin G and azurocidin. The primary function of these neutrophil-derived serine proteases is thought to be degradation of extracellular proteins at sites of inflammation, but excessive or prolonged proteolytic activity may cause harmful effects in the body.

Anti-neutrophil cytoplasmic antibodies (ANCA) are autoantibodies directed against constituents of neutrophil cytoplasm. Two main types of ANCA are present in patients with ANCA-associated systemic vasculitis: cANCA (cytoplasmic pattern in indirect immunofluorescence test, IIF), which target PR3, and pANCA (perinuclear), which are directed against MPO.

cANCA/PR3-ANCA exhibit high specificity as well as sensitivity for Wegeners Granulomatosis, a necrotizing vascultis syndrome, and are much less frequently found in Churg-Strauss Syndrome and Microscopic Polyangiitis. In active Wegener the prevalence of this antibody is almost 90%, decreasing to 30-40% during remission. There is consensus in the literature that diagnostic specificity is optimally improved by combining IIF on neutrophils together with standardized PR3 ELISA. Presently PR3-ANCA is detected by both traditional direct and capture ELISAs; very recently a so-called anchor ELISA (using a bridging molecule to prevent direct adhesion on the plastic surface and therefore preserving all epitopes for binding with ANCA) as well as multiplex systems (e.g. measuring PR3, MPO and GBM autoantibodies in one tube) have also been described.

The close relationship between ANCA and ANCA associated vasculitis, in particular with disease activity, has suggested that ANCA may be pathogenic. Interestingly the parenchymal pneumocytes and macrophages, and not the neutrophils, express PR3 most strongly and may, for instance, contribute to lung damage in patients with WG via direct interaction with ANCA. In this view vascular damage is caused by ANCA-activated leukocytes; whether endothelial damage can arise by direct pathological action of ANCA on endothel cell surface has been speculated for years, but is not rigorously proven.

Human native proteinase 3 (PR3) by DIARECT is purified from polymorphonuclear leukocytes of peripheral human blood.

 

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           韋格納肉芽腫病最出名的抗中性粒細胞胞漿抗體(ANCA)是在壞死性小血管炎中發現的。這些抗體大部分屬于IgG類型,在乙醇固定的中性粒細胞和單核細胞的間接免疫熒光反應中有很明顯的陽性反應,它們的主要靶點是蛋白酶3(PR3)(即是所謂的胞漿型cANCA)和髓過氧化物酶(MPO)??顾柽^氧化物酶抗體在大多數的血清中發現,即產生了核周型pANCA。大部分的血清通過間接免疫熒光反應表現出pANCA模型但并不包含MPO抗體??筆R3特異性抗體是中性粒細胞特異性自身抗體相關脈管炎疾病,特別是韋格納豆芽腫病的一種敏感的和特異性的標記物。在臨床上,如果檢出抗MPO抗體,那么很可能是壞死性脈管炎或原發性的微量免疫壞死性和新月體腎小球腎炎。如今,抗中性粒細胞細胞質抗體在系統性炎癥疾病的臨床診斷中一直在增加。對于它們的描述大概要回到27年前,它們在常規的脈管炎檢測中變成了一種重要的標準?,F在建立的ANCA確定的方法是人中性粒細胞的間接性免疫熒光反應和抗原特異的ELISA,線性分析或者多通道分析。    蛋白酶3是在嗜天青中性粒細胞中的一種中性絲氨酸蛋白酶,由228個氨基酸組成,分子量為29-33kDa(不同糖基化類型有所不同)。它實際上與中性白細胞彈性蛋白酶,組織蛋白酶G,天青素有序列同源性。這些中性粒細胞它主要的功能就是降解炎癥部位的細胞外蛋白。但是過量或拖延的蛋白水解活性可能會對機體有害??怪行粤0准毎|抗體(ANCA)是直接抗中性粒白細胞胞質組分中。在ANCA相關的系統性血管炎患者中發現了兩種主要的ANCA :cANCA(間接免疫熒光檢測IIF的胞質類型),靶向PR3抗原;pANCA(核周),靶向MPO抗原。cANCA/PR3-ANCA對韋格納肉芽腫病和壞死性脈管炎有很高的特異性和敏感性,但是在顯微鏡下型多血管炎和丘施二氏綜合征患者中不常見。該抗體在活動性韋格納肉芽腫病患者中的檢出率幾乎為90%,當癥狀緩解后降至30-40%。目前已有共識。該病的診斷最好將中性粒白細胞間接免疫熒光檢測(IIF)和標準化的PR3 ELISA檢測相結合。之前,檢測PR3-ANCA可以通過傳統直接法ELISA也可以通過捕獲法ELISA。目前,還可用錨定法ELISA(通過搭橋分子阻止蛋白與固相表面的直接結合,從而是所有的表位都結合到ANCA)和多通道檢測系統(如在一個管子中同時檢測PR3,MPO和GBM自身抗體)檢測。 ANCA和ANCA相關血管炎,尤其是疾病活動性之間的密切聯系,表明ANCA可能是致病的。有趣的是實質肺細胞和巨噬細胞,而不是中性粒細胞,強烈表達PR3,并可能比方說通過與ANCA的直接相互作用促成WG病人的肺損傷。從這種觀點來看,血管損傷是由ANCA激活的白細胞引起的,內皮損傷是否會通過ANCA在內皮細胞表面的直接病理活動而加重,這個問題已經爭論了很多年,至今仍沒有一個準確的定論。 DIARECT生產的人天然蛋白酶3(PR3)是從人外周血中的多形核白細胞中純化的。

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