中文名称  穿孔素抗体说明书 

英文名称  Anti-Perforin

别    名 Cytolysin; FLH2; HPLH2; Lymphocyte pore forming protein; Lymphocyte pore-forming protein; MGC65093; P1; PERF_HUMAN; Perforin 1; Perforin 1 precursor; Perforin 1 preforming protein; Perforin-1; PFP; PGFL; PIGF; PIGF-2; PLGF; Pore forming protein; PRF 1; PRF1; SHGC-10760.

产品属性：

穿孔素抗体说明书 浓    度  1mg/1ml

规 格  0.1ml/100μg 0.2ml/200μg

抗体来源  Rabbit

克隆类型   polyclonal

交叉反应   Human, Mouse, Rat, Horse

产品类型   一抗  

研究领域    细胞生物 淋巴细胞 t-淋巴细胞

蛋白分子量  predicted molecular weight: 59kDa 

性    状   Lyophilized or Liquid

免 疫 原  KLH conjugated synthetic peptide derived from human Perforin

亚    型  IgG

纯化方法   affinity purified by Protein A

储 存 液   Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4

穿孔素抗体说明书 产品应用   WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500

（石蜡切片需做抗原修复） 

 not yet tested in other applications.

 optimal dilutions/concentrations should be determined by the end user.  

保存条件  Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. 

Important Note  This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 

产品介绍 The major defense of the body against virus-infected and tumorigenic cells is cytotoxic T lymphocyte (CTL)-mediated cytotoxicity, which also plays a role in autoimmune diseases and transplant rejection. During CTL-mediated cytotoxicity, CTL granules containing perforin are exocytosed. Perforin is a pore-forming protein that facilitates the entry of cytotoxic serine proteases, such as granzymes, into target cells by forming transmembrane channels in target cell membranes. Perforin is primarily expressed in cytotoxic T lymphocytes (CTL) and natural killer (NK) cells, but has also been observed in an astrocyte population of the human brain. It has been shown that abrogation of perforin function by Ca2+-complexing agents leads to decreased levels of necrosis, demonstrating that both necrosis and apoptosis contribute to CTL-mediated cytotoxicity. Perforin activity has been shown to be induced by IL-2, IL-3, IL-4, IL-6 and to a lesser degree, TNF and IFN-γ.

Function : 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.

Subunit : Monomer, as sobluble protein. Homooligomer. Oligomerization is required for pore formation.

Subcellular Location : 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.

DISEASE : 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.

Similarity : Belongs to the complement C6/C7/C8/C9 family.

Contains 1 C2 domain.

Contains 1 EGF-like domain.

Contains 1 MACPF domain.

Database links : UniProtKB/Swiss-Prot: P14222.1

实验流程：

全、新、优、品、好四大特点：

穿孔素抗体说明书 全：公司提供上万种产品，涵盖了生物试剂，elisa试剂盒，标准品，培养基，原装耗材，抗体、培养基、ATCC细胞等，基本上各种科研所需产品在我司都能找到。

新：产品更新速度较快，基本上每周都有新产品出现。

优：产品质量好，投诉比较少。

好：我公司具有优质的技术团队，产品一旦售出，实验过程中遇到困难可提供在线技术咨询。使您使用产品时没有任何的后顾之忧。

技术外包服务:

★穿孔素抗体说明书 分子生物学：质粒抽提、PCR、Q-PCR、RT-PCR、分子生物学：基因合成、引物合成、基因测序、载体构建等

★蛋白工程：原核、哺乳动物蛋白表达系统等

★病毒包装：腺病毒、慢病毒等

★抗体工程：磁珠分选、病理染色、WB、ELISA、IP、IF、IHC、FACS、Confocal等等

★细胞工程：细胞表型分析(凋亡、增殖、周期、迁移、侵袭、修复、克隆形成)、细胞培养、细胞膜制备、稳定细胞株构建、细胞RNAi技术等等。

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