Perforin
Perforin-1 | ||
---|---|---|
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
- ↑ UniProt entry
- ↑ perforin. In: Online Mendelian Inheritance in Man . (English)