Perforin

Perforin
Perforin-1

Vorhandene Strukturdaten: 2qp2
Masse/Länge Primärstruktur 534 Aminosäuren
Sekundär- bis Quartärstruktur multipass Membranprotein
Bezeichner
Gen-Namen PRF1; FLH2; HPLH2; MGC65093; P1; PFP
Externe IDs OMIM170280 UniProtP14222   MGI97551
Transporter-Klassifikation
TCDB 1.C.39.2.1
Bezeichnung Komplementprotein C9-Familie
Vorkommen
Homologie-Familie Perforin-1
Übergeordnetes Taxon Euteleostomi

Perforin (PRF1) ist ein cytolytisches Protein, welches in Granula der cytotoxischen T-Zellen (CD8 positiven T-Lymphocyten) und NK-Zellen gefunden werden kann. Bei der Degranulation perforiert es die Zellmembran der Zielzelle und formt eine Pore. Durch diese Pore dringen Granzyme B in die Zielzelle ein und lösen eine Apoptose aus.

Mutationen im PRF1-Gen können hämophagozytische Lymphohistiozytose Typ 2 (FHL2) verursachen.[1][2]

Literatur

  • Pipkin ME, Lieberman J: Delivering the kiss of death: progress on understanding how perforin works. In: Curr. Opin. Immunol.. 19, Nr. 3, Juni 2007, S. 301–8. doi:10.1016/j.coi.2007.04.011. PMID 17433871.
  • Kurschus FC, Fellows E, Stegmann E, Jenne DE: Granzyme B delivery via perforin is restricted by size, but not by heparan sulfate-dependent endocytosis. In: Proc. Natl. Acad. Sci. U.S.A.. 105, Nr. 37, September 2008, S. 13799–804. doi:10.1073/pnas.0801724105. PMID 18772390. Volltext bei PMC: 2544534.
  • Bolitho P, Voskoboinik I, Trapani JA, Smyth MJ: Apoptosis induced by the lymphocyte effector molecule perforin. In: Curr. Opin. Immunol.. 19, Nr. 3, Juni 2007, S. 339–47. doi:10.1016/j.coi.2007.04.007. PMID 17442557.

Einzelnachweise

  1. UniProt-Eintrag
  2. Perforin bei Online Mendelian Inheritance in Man

Weblinks


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  • Perforin — 1 (pore forming protein), also known as PRF1, is a human gene.cite web | title = Entrez Gene: PRF1 perforin 1 (pore forming protein)| url = http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene Cmd=ShowDetailView TermToSearch=5551| accessdate = ]… …   Wikipedia

  • perforin pathway — The cytotoxic pathway that uses perforin protein, which polymerizes to form membrane pores that help destroy cells during cell mediated cytotoxicity. Perforin is produced by cytotoxic T cells and NK cells and stored in granules that are released… …   Dictionary of microbiology

  • perforin — per·fo·rin (pûrʹfər ĭn) n. A protein in killer cells and natural killer cells that causes lysis of target cells on contact.   [perforate + in.] * * * …   Universalium

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  • perforin — A protein found in the cytoplasmic granules of both T cytotoxic lymphocytes and natural killer cells. This protein is implicated in target cell lysis by the above cells. [L. per foro, to bore, pierce, + in] * * * per·for·in (purґfə rin) a… …   Medical dictionary

  • perforin — [ pə:fərɪn] noun Biochemistry a protein, released by killer cells of the immune system, which destroys targeted cells by creating lesions like pores in their membranes. Origin 1980s: from perforate + in1 …   English new terms dictionary

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  • Granzyme — Granzymes are serine proteases that are released by cytoplasmic granules within cytotoxic T cells and natural killer cells. Their purpose is to induce apoptosis within virus infected cells, thus destroying them.cite journal |author=Bots M, Medema …   Wikipedia

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