穿孔素(英語:Perforin)是人體內一類由PRF1基因编码的蛋白質[2][3][4]。
事实速览 穿孔素1 Perforin 1, 标识 ...
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穿孔素是一種由细胞毒性T细胞或自然殺傷細胞分泌的細胞溶解蛋白質。它們會插入靶細胞的細胞膜中,並形成孔隙。穿孔素的MACPF域能夠促使細胞溶解[5]。該域與革蘭氏陽性菌的膽固醇依賴性溶細胞素有一定的同源型[1]。
穿孔素在結構上和功能上與補體成分9(C9)具有一些共同點。比如說,穿孔素可以像補體成分9那樣在細胞膜上形成能夠非特異性地裂解多種靶細胞的孔洞。穿孔素是促細胞溶解顆粒中的一種主要促細胞裂解蛋白,在由T細胞和天然殺傷細胞介導的細胞溶解中起關鍵作用[4]。它通過在細胞膜上產生孔洞,從而讓鈣離子大量流入細胞,使得靶細胞啓動細胞膜的修復機制。上述的修復機制可讓穿孔素和顆粒酶進入早期內涵體[6]。
有缺陷的PRF1等位基因純合會導致2型噬血細胞综合癥,一種罕見的致死性幼年期常染色體隱性遺傳病[4]。
數據表明,穿孔素可與鈣網蛋白發生蛋白質交互作用[7]。
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