Perforin 1
Sign in to saveAlso known as perforin 1 (pore forming protein), PFP, lymphocyte pore-forming protein, cytolysin, lymphocyte pore forming protein, perforin-1, PRF1, perforin
Perforin-1 (PRF) is a pore-forming protein encoded in humans by the PRF1 gene. It is stored in the secretory granules of cytotoxic T lymphocytes (CTLs) and natural killer (NK) cells, collectively known as cytotoxic lymphocytes (CLs). Upon activation, these cells release perforin to form pores in the membranes of target cells, enabling the entry of granzymes that trigger apoptosis. Perforin is therefore a central effector molecule of the immune system, essential for the elimination of virus-infected and transformed cells. Mutations in PRF1 that impair perforin expression or function are associa
Protein · UniProt
Perforin-1
- Gene
- PRF1
- Organism
- Homo sapiens (Human)
- Length
- 555 aa
- Molecular mass
- 61,377 Da
- Evidence
- 1: Evidence at protein level
Pore-forming protein that plays a key role in granzyme-mediated programmed cell death, and in defense against virus-infected or neoplastic cells (PubMed:20889983, PubMed:21037563, PubMed:24558045, PubMed:9058810, PubMed:9164947). Plays an important role in killing other cells that are recognized as non-self by the immune system, e.g. in transplant rejection or some forms of autoimmune disease (PubMed:9058810). Can insert into the membrane of target cells in its calcium-bound form, oligomerize and form large pores (PubMed:20889983, PubMed:21037563). Promotes cytolysis and apoptosis of target…
Swiss-Prot (reviewed) · via UniProt
Clinical Trials
2 registered- PHASE2TERMINATEDAnalyzing Childhood Recall Antigens in Patients With Pancreatic CancerAlbert Einstein College of Medicine · NCT03848182
- COMPLETEDGranzymes and Perforin at the Onset of Chronic Obstructive Pulmonary Disease (COPD) ExacerbationsUniversity of Thessaly · NCT00883701
~7 min read
Encyclopedic overview
15 sectionsContents
- Discovery
- Structure
- Function
- Clinical significance
- Familial hemophagocytic lymphohistiocytosis
- Perforinopathy
- Acute
- Sub-acute
- Chronic
- Cancer
- Interactions
- See also
- References
- Further reading
- External links
Perforin-1 (PRF) is a pore-forming protein encoded in humans by the PRF1 gene. It is stored in the secretory granules of cytotoxic T lymphocytes (CTLs) and natural killer (NK) cells, collectively known as cytotoxic lymphocytes (CLs). Upon activation, these cells release perforin to form pores in the membranes of target cells, enabling the entry of granzymes that trigger apoptosis. Perforin is therefore a central effector molecule of the immune system, essential for the elimination of virus-infected and transformed cells. Mutations in PRF1 that impair perforin expression or function are associated with familial hemophagocytic lymphohistiocytosis (FHL) and related immune dysregulation syndromes, a spectrum of conditions sometimes collectively referred to as perforinopathies.
== Discovery == Perforin was initially discovered in 1983 and subsequently cloned from an expression library in 1988 using anti-complement C9 antibody cross-reactivity. A sequence comparison showed a notable resemblance between the two proteins in a specific central region, termed the 'membrane attack complex/perforin' (MACPF) domain.
Excerpted from Wikipedia’s “Perforin 1” article, available under the CC BY-SA 4.0 licence.