穿孔素抗体
规格：1mg/1ml
英文名： 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; PG
分子量： 59kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human Perforin
交叉反应：Human,
细胞定位：细胞浆 细胞膜
穿孔素抗体产品介绍：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: 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: 穿孔素抗体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. Post-translational modifications: N-glycosylated. 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穿孔素抗体 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. Gene ID: 5551 Database links: Entrez Gene: 5551 Human Entrez Gene: 18646 Mouse Entrez Gene: 50669 Rat Omim: 170280 Human SwissProt: P14222 Human SwissProt: P10820 Mouse SwissProt: P35763 Rat Unigene: 2200 Human Unigene: 240313 Mouse Unigene: 11206 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
产穿孔素抗体品应用：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.
研究领域：细胞生物 **细胞 t-**细胞
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid