Perforin mouse mAb(ABT-P1)

    • Catalog No.:YM6760
    • Applications:IHC-p
    • Reactivity:Human
      • Gene Name:
      • PRF1 PFP
      • Protein Name:
      • Perforin
      • Human Gene Id:
      • 5551
      • Human Swiss Prot No:
      • P14222
      • Immunogen:
      • Synthesized peptide derived from human Perforin
      • Specificity:
      • This antibody detects endogenous levels of human Perforin, TRIS-EDTA of pH9.0 was used for Heat-induced epitope retrieval (HIER)
      • Formulation:
      • Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
      • Source:
      • Mouse
      • Dilution:
      • IHC-p 1:50-500
      • Purification:
      • The antibody was affinity-purified from mouse ascites by affinity-chromatography using specific immunogen.
      • Concentration:
      • 1 mg/ml
      • Storage Stability:
      • -20°C/1 year
      • Other Name:
      • Perforin-1 (P1) (Cytolysin) (Lymphocyte pore-forming protein) (PFP)
      • Background:
      • 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],
      • 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 lys
      • Products Images
      • Immunohistochemical analysis of paraffin-embedded human Spleen. 1, Antibody was diluted at 1:200(4°,overnight). 2, TRIS-EDTA of pH9.0 was used for antigen retrieval. 3,Secondary antibody was diluted at 1:200(room temperature, 30min).