Anti-Perforin 抗体 [B-D48], prediluted (FITC) (ab86318)

製品の概要

• 製品名Anti-Perforin antibody [B-D48], prediluted (FITC)
  Perforin 一次抗体 製品一覧
• 製品の詳細
  Mouse monoclonal [B-D48] to Perforin, prediluted (FITC)
• 標識FITC. Ex: 493nm, Em: 528nm
• アプリケーション適用あり: Flow Cytmore details
• 種交差性
  交差種: Human
  
• 免疫原

  Natural Human Perforin

製品の特性

• 製品の状態Liquid
• 保存方法Shipped at 4°C. Store at +4°C.
• バッファーPreservative: 0.1% Sodium Azide
  Constituents: 5% BSA, PBS
• Concentration information loading...
• 精製度Ion Exchange Chromatography
• ポリ/モノモノクローナル
• クローン名B-D48
• ミエローマx63-Ag8.653
• アイソタイプIgG1
• 研究分野
  • Immunology
  • Adaptive Immunity
  • T Cells
  • Cytotoxic Cells

  • Immunology
  • Innate Immunity
  • Complement
  • Other

関連製品

• Compatible Secondaries
  • Goat Anti-Mouse IgG H&L (Alexa Fluor® 488) (ab150113)
  • Goat Anti-Mouse IgG H&L (HRP) (ab205719)

アプリケーション

ターゲット情報

• 機能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.
• 関連疾患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.
• 配列類似性Belongs to the complement C6/C7/C8/C9 family.
  Contains 1 C2 domain.
  Contains 1 EGF-like domain.
  Contains 1 MACPF domain.
• ドメインThe C2 domain mediates calcium-dependent binding to lipid membranes. A subsequent conformation change leads to membrane insertion of beta-hairpin structures and pore formation. The pore is formed by transmembrane beta-strands.
• 翻訳後修飾N-glycosylated.
• 細胞内局在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.

• Target information above from: UniProt accession P14222 The UniProt Consortium
  The Universal Protein Resource (UniProt) in 2010
  Nucleic Acids Res. 38:D142-D148 (2010) .

  Information by UniProt
• 参照データベース
  • Entrez Gene: 5551 Human
  • Omim: 170280 Human
  • SwissProt: P14222 Human
  • Unigene: 2200 Human
• 別名
  • Cytolysin antibody
  • FLH2 antibody
  • HPLH2 antibody

  • Lymphocyte pore-forming protein antibody
  • P1 antibody
  • PERF_HUMAN antibody
  • perforin 1 (pore forming protein) antibody
  • Perforin 1 antibody
  • Perforin-1 antibody
  • PFP antibody
  • PGFL antibody
  • PIGF antibody
  • PIGF-2 antibody
  • PLGF antibody
  • Pore forming protein antibody
  • prf1 antibody
  • SHGC-10760 antibody

  see all