ITT5792
ITT5792
- Catalog: ITT5792
- Gene/Protein: PRF1
- Product Description: Immunotag™ Perforin 1 Polyclonal Antibody
385.0000
Price in reward points: 385
| Antibody Specification | |
| Datasheet | 
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| Target Protein | Perforin 1 |
| Clonality | Polyclonal |
| Storage/Stability | -20°C/1 year |
| Application | WB,IHC,ELISA |
| Recommended Dilution | WB 1:500-2000, IHC 1:50-200, ELISA 1:10000-20000 |
| Concentration | 1 mg/ml |
| Reactive Species | Human |
| Host Species | Rabbit |
| Immunogen | The antiserum was produced against synthesized peptide derived from the C-terminal region of human PRF1. AA range:451-500 |
| Specificity | Perforin 1 Polyclonal Antibody detects endogenous levels of Perforin 1 |
| Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen |
| Form | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Gene Name | PRF1 |
| Accession No. | P14222 P10820 |
| Alternate Names | Perforin-1 (P1) (Cytolysin) (Lymphocyte pore-forming protein) (PFP) |
| Description | perforin 1(PRF1) Homo sapiens The protein encoded by this gene has structural and functional similarities to complement component 9 (C9). Like C9, this protein creates transmembrane tubules and is capable of lysing non-specifically a variety of target cells. This protein is one of the main cytolytic proteins of cytolytic granules, and it is known to be a key effector molecule for T-cell- and natural killer-cell-mediated cytolysis. Defects in this gene cause familial hemophagocytic lymphohistiocytosis type 2 (HPLH2), a rare and lethal autosomal recessive disorder of early childhood. Alternative splicing results in multiple transcript variants encoding the same protein. [provided by RefSeq, Jul 2008], |
| Cell Pathway/ Category | Natural killer cell mediated cytotoxicity,Type I diabetes mellitus,Autoimmune thyroid disease,Allograft rejection,Graft-versus-host disease,Viral myocarditis, |
| Protein Expression | Liver,Natural killer cell,Spleen, |
| Subcellular Localization | extracellular region,plasma membrane,membrane,integral component of membrane,cytoplasmic, membrane-bounded vesicle,endosome lumen,cytolytic granule, |
| Protein Function | disease:Defects in PRF1 are the cause of familial hemophagocytic lymphohistiocytosis 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.,function:In the presence of calcium, perforin polymerizes into transmembrane tubules and is capable of lysing non-specifically a variety of target cells.,induction:Repressed by contact with target cells.,online information:Perforin entry,online information:PRF1 mutation db,similarity:Belongs to the complement C6/C7/C8/C9 family.,similarity:Contains 1 C2 domain.,similarity:Contains 1 EGF-like domain.,similarity:Contains 1 MACPF domain.,subcellular location:Cytoplasmic granules of cytolytic T-lymphocytes., |
| Usage | For Research Use Only! Not for diagnostic or therapeutic procedures. |
| Material Safety Data Sheet | |
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