Immunogen
Synthesized peptide derived from human Perforin
Specificity
The antibody can specifically recognize human Perforin protein. In western blotting of Jurkat cell lysate, the antibody can label a 61 kDa band corresponding to Perforin.
Source
Mouse, Monoclonal/IgG1, Kappa
Formulation
PBS, pH7.2, 0.03% Porcolin 300, containing stabilizing protein
Dilution rate
IHC-p 1:200-400,WB: 500-1000
Purification process (Immunogen)
The antibody was affinity-purified from mouse ascites by affinity-chromatography using specific immunogen.
Background
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
Protein name
Cytolysin;FLH2;HPLH2;Lymphocyte pore-forming protein;P1;PERF_HUMAN;perforin 1 (pore forming protein);Perforin 1;Perforin-1;PFP;PGFL;PIGF;PIGF-2;PLGF;Pore forming protein;prf1;SHGC-10760
Other name
Cytolysin;FLH2;HPLH2;Lymphocyte pore-forming protein;P1;PERF_HUMAN;perforin 1 (pore forming protein);Perforin 1;Perforin-1;PFP;PGFL;PIGF;PIGF-2;PLGF;Pore forming protein;prf1;SHGC-10760
Fields
>>Apoptosis;>>Natural killer cell mediated cytotoxicity;>>Type I diabetes mellitus;>>Autoimmune thyroid disease;>>Allograft rejection;>>Graft-versus-host disease;>>Viral myocarditis
Human protein sequence Database
P14222
Mouse protein sequence database
P10820
Rat protein sequence database
P35763
Cellular localization
Cytoplasmic, Membranous
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