• 靶点：
• Perforin

• 简介：
• >>Apoptosis;>>Natural killer cell mediated cytotoxicity;>>Type I diabetes mellitus;>>Autoimmune thyroid disease;>>Allograft rejection;>>Graft-versus-host disease;>>Viral myocarditis

• 基因名称：
• PRF1

• 蛋白名称：
• 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

• Human Swiss Prot No：
• P14222

• Mouse Swiss Prot No：
• P10820

• Rat Swiss Prot No：
• P35763

• 免疫原：
• Synthesized peptide derived from human Perforin AA range:1-100

• 特异性：
• This antibody detects endogenous levels of Perforin

• 组成：
• PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA

• 来源：
• Monoclonal, Rabbit IgG1, Kappa

• 稀释：
• IHC 1:100-500, WB 1:500-1000, ELISA 1:5000-20000

• 纯化工艺：
• Recombinant Expression and Affinity purified

• 储存：
• -15°C to -25°C/1 year(Do not lower than -25°C)

• 其他名称：
• 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

• 分子量：
• 61kD

• 背景：
• 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],

• 功能：
• 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

• 细胞定位：
• Cytoplasmic, Membranous

• 组织表达：
• Spleen

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