^ 1.01.1Rosado CJ, Buckle AM, Law RH, Butcher RE, Kan WT, Bird CH, Ung K, Browne KA, Baran K, Bashtannyk-Puhalovich TA, Faux NG, Wong W, Porter CJ, Pike RN, Ellisdon AM, Pearce MC, Bottomley SP, Emsley J, Smith AI, Rossjohn J, Hartland EL, Voskoboinik I, Trapani JA, Bird PI, Dunstone MA, Whisstock JC. A common fold mediates vertebrate defense and bacterial attack. Science. 2007, 317 (5844): 1548–51. PMID 17717151. doi:10.1126/science.1144706.
^Fink TM, Zimmer M, Weitz S, Tschopp J, Jenne DE, Lichter P. Human perforin (PRF1) maps to 10q22, a region that is syntenic with mouse chromosome 10. Genomics. Sep 1992, 13 (4): 1300–2. PMID 1505959. doi:10.1016/0888-7543(92)90050-3.
^Shinkai Y, Yoshida MC, Maeda K, Kobata T, Maruyama K, Yodoi J, Yagita H, Okumura K. Molecular cloning and chromosomal assignment of a human perforin (PFP) gene. Immunogenetics. Jan 1990, 30 (6): 452–7. PMID 2592021. doi:10.1007/BF02421177.
^Tschopp J, Masson D, Stanley KK. Structural/functional similarity between proteins involved in complement- and cytotoxic T-lymphocyte-mediated cytolysis. Nature. 1986, 322 (6082): 831–4. PMID 2427956. doi:10.1038/322831a0.
^Thiery J, Keefe D, Boulant S, Boucrot E, Walch M, Martinvalet D, Goping IS, Bleackley RC, Kirchhausen T, Lieberman J. Perforin pores in the endosomal membrane trigger the release of endocytosed granzyme B into the cytosol of target cells. Nat. Immunol. 2011, 12 (8): 770–7. PMC 3140544. PMID 21685908. doi:10.1038/ni.2050.
^Andrin C, Pinkoski MJ, Burns K; et al. Interaction between a Ca2+-binding protein calreticulin and perforin, a component of the cytotoxic T-cell granules. Biochemistry. July 1998, 37 (29): 10386–94. PMID 9671507. doi:10.1021/bi980595z. 引文格式1维护：显式使用等标签 (link)
拓展閱讀编辑
Trapani JA. Target cell apoptosis induced by cytotoxic T cells and natural killer cells involves synergy between the pore-forming protein, perforin, and the serine protease, granzyme B. Australian and New Zealand journal of medicine. 1996, 25 (6): 793–9. PMID 8770355. doi:10.1111/j.1445-5994.1995.tb02883.x.
Peitsch MC, Amiguet P, Guy R; et al. Localization and molecular modelling of the membrane-inserted domain of the ninth component of human complement and perforin. Mol. Immunol. 1990, 27 (7): 589–602. PMID 2395434. doi:10.1016/0161-5890(90)90001-G. 引文格式1维护：显式使用等标签 (link)
Young JD, Hengartner H, Podack ER, Cohn ZA. Purification and characterization of a cytolytic pore-forming protein from granules of cloned lymphocytes with natural killer activity. Cell. 1986, 44 (6): 849–59. PMID 2420467. doi:10.1016/0092-8674(86)90007-3.
Young JD, Cohn ZA, Podack ER. The ninth component of complement and the pore-forming protein (perforin 1) from cytotoxic T cells: structural, immunological, and functional similarities. Science. 1986, 233 (4760): 184–90. PMID 2425429. doi:10.1126/science.2425429.
Lichtenheld MG, Podack ER. Structure of the human perforin gene. A simple gene organization with interesting potential regulatory sequences. J. Immunol. 1990, 143 (12): 4267–74. PMID 2480391.
Shinkai Y, Takio K, Okumura K. Homology of perforin to the ninth component of complement (C9). Nature. 1988, 334 (6182): 525–7. PMID 3261391. doi:10.1038/334525a0.
Lichtenheld MG, Olsen KJ, Lu P; et al. Structure and function of human perforin. Nature. 1988, 335 (6189): 448–51. PMID 3419519. doi:10.1038/335448a0. 引文格式1维护：显式使用等标签 (link)
Goebel WS, Schloemer RH, Brahmi Z. Target cell-induced perforin mRNA turnover in NK3.3 cells is mediated by multiple elements within the mRNA coding region. Mol. Immunol. 1996, 33 (4–5): 341–9. PMID 8676885. doi:10.1016/0161-5890(95)00155-7.
Nöske K, Bilzer T, Planz O, Stitz L. Virus-Specific CD4+ T Cells Eliminate Borna Disease Virus from the Brain via Induction of Cytotoxic CD8+ T Cells. J. Virol. 1998, 72 (5): 4387–95. PMC 109669. PMID 9557729.
Andrin C, Pinkoski MJ, Burns K; et al. Interaction between a Ca2+-binding protein calreticulin and perforin, a component of the cytotoxic T-cell granules. Biochemistry. 1998, 37 (29): 10386–94. PMID 9671507. doi:10.1021/bi980595z. 引文格式1维护：显式使用等标签 (link)
Yu CR, Ortaldo JR, Curiel RE; et al. Role of a STAT binding site in the regulation of the human perforin promoter. J. Immunol. 1999, 162 (5): 2785–90. PMID 10072525. 引文格式1维护：显式使用等标签 (link)
Stepp SE, Dufourcq-Lagelouse R, Le Deist F; et al. Perforin gene defects in familial hemophagocytic lymphohistiocytosis. Science. 1999, 286 (5446): 1957–9. PMID 10583959. doi:10.1126/science.286.5446.1957. 引文格式1维护：显式使用等标签 (link)
Takahashi T, Nieda M, Koezuka Y; et al. Analysis of human V alpha 24+ CD4+ NKT cells activated by alpha-glycosylceramide-pulsed monocyte-derived dendritic cells. J. Immunol. 2000, 164 (9): 4458–64. PMID 10779745. doi:10.4049/jimmunol.164.9.4458. 引文格式1维护：显式使用等标签 (link)
Badovinac VP, Tvinnereim AR, Harty JT. Regulation of antigen-specific CD8+ T cell homeostasis by perforin and interferon-gamma. Science. 2000, 290 (5495): 1354–8. PMID 11082062. doi:10.1126/science.290.5495.1354.
Göransdotter Ericson K, Fadeel B, Nilsson-Ardnor S; et al. Spectrum of Perforin Gene Mutations in Familial Hemophagocytic Lymphohistiocytosis. Am. J. Hum. Genet. 2001, 68 (3): 590–7. PMC 1274472. PMID 11179007. doi:10.1086/318796. 引文格式1维护：显式使用等标签 (link)
Clementi R, zur Stadt U, Savoldi G; et al. Six novel mutations in the PRF1 gene in children with haemophagocytic lymphohistiocytosis. J. Med. Genet. 2002, 38 (9): 643–6. PMC 1734943. PMID 11565555. doi:10.1136/jmg.38.9.643. 引文格式1维护：显式使用等标签 (link)
Ambach A, Bonnekoh B, Gollnick H. Perforin granule release from cytotoxic lymphocytes ex vivo is inhibited by ciclosporin but not by methotrexate. Skin Pharmacol. Appl. Skin Physiol. 2001, 14 (5): 249–60. PMID 11586066. doi:10.1159/000056355.
穿孔素, 英語, perforin, 是人體內一類由prf1基因编码的蛋白質, 1perforin, 1一個包含plu, macpf蛋白的macpf域結構pdb, 2qp2, macpf域用青色, 粉紅色表示, 棱鏡域則是用橘色表示, 兩個鈣原子則是用灰色表示标识代号prf1, flh2, hplh2, pfn1, pfp扩展标识遗传学, 170280, 鼠基因, 97551, 同源基因, 3698, chembl, 5480, genecards, prf1, gene基因本体论描述分子功能, calcium, . 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 穿孔素1Perforin 1一個包含Plu MACPF蛋白的MACPF域結構PDB 2QP2 1 MACPF域用青色 粉紅色表示 而 棱鏡域則是用橘色表示 兩個鈣原子則是用灰色表示标识代号PRF1 FLH2 HPLH2 P1 PFN1 PFP扩展标识遗传学 170280 鼠基因 97551 同源基因 3698 ChEMBL 5480 GeneCards PRF1 Gene基因本体论描述分子功能 calcium ion binding protein binding wide pore channel activity细胞成分 extracellular region plasma membrane membrane integral component of membrane cytoplasmic membrane bounded vesicle endosome lumen cytolytic granule生物过程 defense response to tumor cell immune response to tumor cell apoptotic process cellular defense response cytolysis protein homooligomerization defense response to virus transmembrane transportSources Amigo QuickGORNA表达模式更多表达数据直系同源体物种人类小鼠Entrez555118646EnsemblENSG00000180644ENSMUSG00000037202UniProtP14222P10820mRNA序列NM 001083116NM 011073蛋白序列NP 001076585NP 035203基因位置Chr 10 72 36 72 36 MbChr 10 61 3 61 3 MbPubMed查询 1 2 查论编 目录 1 功能 2 臨床意義 3 相互作用 4 參見 5 參考 6 拓展閱讀 7 外部連結功能 编辑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 英语 Cytolysis 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 英语 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 臨床意義 编辑有缺陷的PRF1等位基因純合會導致2型噬血細胞综合癥 英语 hemophagocytic lymphohistiocytosis 一種罕見的致死性幼年期常染色體隱性遺傳病 4 相互作用 编辑數據表明 穿孔素可與鈣網蛋白 英语 calreticulin 發生蛋白質交互作用 7 參見 编辑顆粒酶 英语 Granzyme 防禦素參考 编辑 1 0 1 1 Rosado CJ Buckle AM Law RH Butcher RE Kan WT Bird CH Ung K Browne KA Baran K Bashtannyk Puhalovich TA Faux NG Wong W Porter CJ Pike RN Ellisdon AM Pearce MC Bottomley SP Emsley J Smith AI Rossjohn J Hartland EL Voskoboinik I Trapani JA Bird PI Dunstone MA Whisstock JC A common fold mediates vertebrate defense and bacterial attack Science 2007 317 5844 1548 51 PMID 17717151 doi 10 1126 science 1144706 Fink TM Zimmer M Weitz S Tschopp J Jenne DE Lichter P Human perforin PRF1 maps to 10q22 a region that is syntenic with mouse chromosome 10 Genomics Sep 1992 13 4 1300 2 PMID 1505959 doi 10 1016 0888 7543 92 90050 3 Shinkai Y Yoshida MC Maeda K Kobata T Maruyama K Yodoi J Yagita H Okumura K Molecular cloning and chromosomal assignment of a human perforin PFP gene Immunogenetics Jan 1990 30 6 452 7 PMID 2592021 doi 10 1007 BF02421177 4 0 4 1 4 2 Entrez Gene PRF1 perforin 1 pore forming protein Tschopp J Masson D Stanley KK Structural functional similarity between proteins involved in complement and cytotoxic T lymphocyte mediated cytolysis Nature 1986 322 6082 831 4 PMID 2427956 doi 10 1038 322831a0 Thiery J Keefe D Boulant S Boucrot E Walch M Martinvalet D Goping IS Bleackley RC Kirchhausen T Lieberman J Perforin pores in the endosomal membrane trigger the release of endocytosed granzyme B into the cytosol of target cells Nat Immunol 2011 12 8 770 7 PMC 3140544 nbsp PMID 21685908 doi 10 1038 ni 2050 Andrin C Pinkoski MJ Burns K et al Interaction between a Ca2 binding protein calreticulin and perforin a component of the cytotoxic T cell granules Biochemistry July 1998 37 29 10386 94 PMID 9671507 doi 10 1021 bi980595z 引文格式1维护 显式使用等标签 link 拓展閱讀 编辑Trapani JA Target cell apoptosis induced by cytotoxic T cells and natural killer cells involves synergy between the pore forming protein perforin and the serine protease granzyme B Australian and New Zealand journal of medicine 1996 25 6 793 9 PMID 8770355 doi 10 1111 j 1445 5994 1995 tb02883 x Peitsch MC Amiguet P Guy R et al Localization and molecular modelling of the membrane inserted domain of the ninth component of human complement and perforin Mol Immunol 1990 27 7 589 602 PMID 2395434 doi 10 1016 0161 5890 90 90001 G 引文格式1维护 显式使用等标签 link Young JD Hengartner H Podack ER Cohn ZA Purification and characterization of a cytolytic pore forming protein from granules of cloned lymphocytes with natural killer activity Cell 1986 44 6 849 59 PMID 2420467 doi 10 1016 0092 8674 86 90007 3 Young JD Cohn ZA Podack ER The ninth component of complement and the pore forming protein perforin 1 from cytotoxic T cells structural immunological and functional similarities Science 1986 233 4760 184 90 PMID 2425429 doi 10 1126 science 2425429 Lichtenheld MG Podack ER Structure of the human perforin gene A simple gene organization with interesting potential regulatory sequences J Immunol 1990 143 12 4267 74 PMID 2480391 Shinkai Y Takio K Okumura K Homology of perforin to the ninth component of complement C9 Nature 1988 334 6182 525 7 PMID 3261391 doi 10 1038 334525a0 Lichtenheld MG Olsen KJ Lu P et al Structure and function of human perforin Nature 1988 335 6189 448 51 PMID 3419519 doi 10 1038 335448a0 引文格式1维护 显式使用等标签 link Goebel WS Schloemer RH Brahmi Z Target cell induced perforin mRNA turnover in NK3 3 cells is mediated by multiple elements within the mRNA coding region Mol Immunol 1996 33 4 5 341 9 PMID 8676885 doi 10 1016 0161 5890 95 00155 7 Noske K Bilzer T Planz O Stitz L Virus Specific CD4 T Cells Eliminate Borna Disease Virus from the Brain via Induction of Cytotoxic CD8 T Cells J Virol 1998 72 5 4387 95 PMC 109669 nbsp PMID 9557729 Andrin C Pinkoski MJ Burns K et al Interaction between a Ca2 binding protein calreticulin and perforin a component of the cytotoxic T cell granules Biochemistry 1998 37 29 10386 94 PMID 9671507 doi 10 1021 bi980595z 引文格式1维护 显式使用等标签 link Yu CR Ortaldo JR Curiel RE et al Role of a STAT binding site in the regulation of the human perforin promoter J Immunol 1999 162 5 2785 90 PMID 10072525 引文格式1维护 显式使用等标签 link Stepp SE Dufourcq Lagelouse R Le Deist F et al Perforin gene defects in familial hemophagocytic lymphohistiocytosis Science 1999 286 5446 1957 9 PMID 10583959 doi 10 1126 science 286 5446 1957 引文格式1维护 显式使用等标签 link Takahashi T Nieda M Koezuka Y et al Analysis of human V alpha 24 CD4 NKT cells activated by alpha glycosylceramide pulsed monocyte derived dendritic cells J Immunol 2000 164 9 4458 64 PMID 10779745 doi 10 4049 jimmunol 164 9 4458 引文格式1维护 显式使用等标签 link Badovinac VP Tvinnereim AR Harty JT Regulation of antigen specific CD8 T cell homeostasis by perforin and interferon gamma Science 2000 290 5495 1354 8 PMID 11082062 doi 10 1126 science 290 5495 1354 Goransdotter Ericson K Fadeel B Nilsson Ardnor S et al Spectrum of Perforin Gene Mutations in Familial Hemophagocytic Lymphohistiocytosis Am J Hum Genet 2001 68 3 590 7 PMC 1274472 nbsp PMID 11179007 doi 10 1086 318796 引文格式1维护 显式使用等标签 link Clementi R zur Stadt U Savoldi G et al Six novel mutations in the PRF1 gene in children with haemophagocytic lymphohistiocytosis J Med Genet 2002 38 9 643 6 PMC 1734943 nbsp PMID 11565555 doi 10 1136 jmg 38 9 643 引文格式1维护 显式使用等标签 link Ambach A Bonnekoh B Gollnick H Perforin granule release from cytotoxic lymphocytes ex vivo is inhibited by ciclosporin but not by methotrexate Skin Pharmacol Appl Skin Physiol 2001 14 5 249 60 PMID 11586066 doi 10 1159 000056355 外部連結 编辑醫學主題詞表 MeSH perforin 取自 https zh wikipedia org w index php title 穿孔素 amp oldid 69141633, 维基百科，wiki，书籍，书籍，图书馆，
