Key features and details
- Rabbit polyclonal to Perforin
- Suitable for: WB
- Reacts with: Mouse, Rat
- Isotype: IgG
保存方法Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.
バッファーPreservative: 0.1% Sodium azide
Concentration information loading...
精製度Protein A purified
Our Abpromise guarantee covers the use of ab7203 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||1/1000. Predicted molecular weight: 70 kDa.|
機能Plays a key role in secretory granule-dependent cell death, and in defense against virus-infected or neoplastic cells. 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. Can insert into the membrane of target cells in its calcium-bound form, oligomerize and form large pores. Promotes cytolysis and apoptosis of target cells by facilitating the uptake of cytotoxic granzymes.
関連疾患Defects in PRF1 are the cause of hemophagocytic lymphohistiocytosis familial type 2 (FHL2) [MIM:603553]; also known as HPLH2. Familial hemophagocytic lymphohistiocytosis (FHL) is a genetically heterogeneous, rare autosomal recessive disorder. It is characterized by immune dysregulation with hypercytokinemia and defective natural killer cell function. The clinical features of the disease include fever, hepatosplenomegaly, cytopenia, hypertriglyceridemia, hypofibrinogenemia, and neurological abnormalities ranging from irritability and hypotonia to seizures, cranial nerve deficits, and ataxia. Hemophagocytosis is a prominent feature of the disease, and a non-malignant infiltration of macrophages and activated T lymphocytes in lymph nodes, spleen, and other organs is also found.
配列類似性Belongs to the complement C6/C7/C8/C9 family.
Contains 1 C2 domain.
Contains 1 EGF-like domain.
Contains 1 MACPF domain.
ドメインThe C2 domain mediates calcium-dependent binding to lipid membranes. A subsequent conformation change leads to membrane insertion of beta-hairpin structures and pore formation. The pore is formed by transmembrane beta-strands.
細胞内局在Cytoplasmic granule lumen. Secreted. Cell membrane. Endosome lumen. Stored in cytoplasmic granules of cytolytic T-lymphocytes and secreted into the cleft between T-lymphocyte and target cell. Inserts into the cell membrane of target cells and forms pores. Membrane insertion and pore formation requires a major conformation change. May be taken up via endocytosis involving clathrin-coated vesicles and accumulate in a first time in large early endosomes.
- Information by UniProt
- Cytolysin antibody
- FLH2 antibody
- HPLH2 antibody
ab7203 は 3 報の論文で使用されています。
- Rothhammer V et al. a4-integrins control viral meningoencephalitis through differential recruitment of T helper cell subsets. Acta Neuropathol Commun 2:27 (2014). WB . PubMed: 24606807
- Kish DD et al. Neutrophil expression of Fas ligand and perforin directs effector CD8 T cell infiltration into antigen-challenged skin. J Immunol 189:2191-202 (2012). WB . PubMed: 22815291
- Mostböck S et al. Enhanced levels of costimulation lead to reduced effector/memory CD8+ T cell functionality. J Immunol 179:3524-34 (2007). WB ; Mouse . PubMed: 17785786