Perforin

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Perforin-1

Existing structural data : 2qp2

Properties of human protein
Mass / length primary structure 534 amino acids
Secondary to quaternary structure multipass membrane protein
Identifier
Gene names PRF1  ; FLH2; HPLH2; MGC65093; P1; PFP
External IDs
Transporter classification
TCDB 1.C.39.2.1
designation Complement protein C9 family
Occurrence
Homology family Perforin-1
Parent taxon Euteleostomi

Perforin ( PRF1 ) is a cytolytic protein that can be found in granules of cytotoxic T cells (CD8 positive T lymphocytes ) and NK cells . During degranulation, it perforates the cell membrane of the target cell and forms a pore. Granzyme B penetrates the target cell through this pore and triggers apoptosis .

Mutations in PRF1 - gene can hemophagocytic lymphohistiocytosis cause type 2 (FHL2).

literature

  • Pipkin ME, Lieberman J: Delivering the kiss of death: progress on understanding how perforin works . In: Curr. Opin. Immunol. . 19, No. 3, June 2007, pp. 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. USA . 105, No. 37, September 2008, pp. 13799-804. doi : 10.1073 / pnas.0801724105 . PMID 18772390 . PMC 2544534 (free full text).
  • Bolitho P, Voskoboinik I, Trapani JA, Smyth MJ : Apoptosis induced by the lymphocyte effector molecule perforin . In: Curr. Opin. Immunol. . 19, No. 3, June 2007, pp. 339-47. doi : 10.1016 / j.coi.2007.04.007 . PMID 17442557 .

Individual evidence

  1. UniProt entry
  2. perforin.  In: Online Mendelian Inheritance in Man . (English)

Web links