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Mouse Anti-PRF1 Recombinant Antibody (Pf-344) (CBMAB-P2768-YC)


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Summary

Host Animal
Mouse
Specificity
Human, Pig
Clone
Pf-344
Antibody Isotype
IgG1
Application
ELISpot, ELISA, FC, WB

Basic Information

Specificity
Human, Pig
Antibody Isotype
IgG1
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
PBS with 0.02% sodium azide
Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at-20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Perforin 1
Introduction
PRF1 is a protein with structural similarities to complement component C9 that is important in immunity. This protein forms membrane pores that allow the release of granzymes and subsequent cytolysis of target cells. Whether pore formation occurs in the plasma membrane of target cells or in an endosomal membrane inside target cells is subject to debate. Mutations in this gene are associated with a variety of human disease including diabetes, multiple sclerosis, lymphomas, autoimmune lymphoproliferative syndrome (ALPS), aplastic anemia, and familial hemophagocytic lymphohistiocytosis type 2 (FHL2), a rare and lethal autosomal recessive disorder of early childhood.
Entrez Gene ID
Human57580
Pig396595
UniProt ID
HumanQ8TCU6
PigF1SUB6
Alternative Names
FLH2; HPLH2; P1; PFN1; PFP
Function
Pore-forming protein that plays a key role in secretory granule-dependent cell death, and in defense against virus-infected or neoplastic cells (PubMed:9058810, PubMed:9164947, PubMed:20889983, PubMed:21037563).
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 cells by facilitating the uptake of cytotoxic granzymes (PubMed:20038786, PubMed:20225066, PubMed:32299851).
Biological Process
Apoptotic processManual Assertion Based On ExperimentTAS:ProtInc
Cellular defense responseManual Assertion Based On ExperimentTAS:ProtInc
CytolysisIEA:UniProtKB-KW
Defense response to tumor cellISS:UniProtKB
Defense response to virusISS:UniProtKB
Immune response to tumor cellISS:UniProtKB
Immunological synapse formationManual Assertion Based On ExperimentIDA:UniProtKB
Positive regulation of killing of cells of another organismManual Assertion Based On ExperimentIDA:UniProtKB
Protein homooligomerizationManual Assertion Based On ExperimentIDA:UniProtKB
T cell mediated cytotoxicityManual Assertion Based On ExperimentIBA:GO_Central
Cellular Location
Cytolytic granule
Secreted
Cell membrane
Endosome lumen
Stored in cytolytic granules of cytolytic T-lymphocytes and secreted into the cleft between T-lymphocyte and target cell (PubMed:20038786).
Inserts into the cell membrane of target cells and forms pores (PubMed:20889983).
Membrane insertion and pore formation requires a major conformation change (PubMed:20889983).
May be taken up via endocytosis involving clathrin-coated vesicles and accumulate in a first time in large early endosomes (PubMed:20038786).
Involvement in disease
Hemophagocytic lymphohistiocytosis, familial, 2 (FHL2):
A rare disorder characterized by immune dysregulation with hypercytokinemia, defective function of natural killer cell, and massive infiltration of several organs by activated lymphocytes and macrophages. The clinical features of the disease include fever, hepatosplenomegaly, cytopenia, and less frequently neurological abnormalities ranging from irritability and hypotonia to seizures, cranial nerve deficits and ataxia.
PTM
N-glycosylated.
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For research use only. Not intended for any clinical use.

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