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 OMIM: 170280 UniProt: P14222   MGI: 97551
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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