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Rabbit Anti-Perforin/BF647 Conjugated antibody (bs-20270R-BF647)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-20270R-BF647
英文名稱 Rabbit Anti-Perforin/BF647 Conjugated antibody
中文名稱 BF647標記的穿孔素抗體
別    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.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 細胞生物  淋巴細胞  t-淋巴細胞  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human, 
產品應用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 59kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Perforin
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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.
產品介紹 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:
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 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:

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.
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