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Rabbit Anti-Niemann Pick C1 Like 1/FITC Conjugated antibody
FITC标记的鞘***脂沉积病C1样蛋白1抗体
| 英文名称 | Anti-Niemann Pick C1 Like 1/FITC |
| 中文名称 | FITC标记的鞘***脂沉积病C1样蛋白1抗体 |
| 别 名 | Niemann Pick C1 like protein 1; Niemann Pick disease type C1 gene like 1; Niemann-Pick C1-like protein 1; NPC1 LIKE 1; NPC1 Niemann Pick disease type C1 gene like 1; NPC1L1; NPC1L1 splice variant; NPCL1_HUMAN; Truncated Niemann Pick C1 like protein 1. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 肿瘤 细胞生物 免疫学 神经生物学 信号转导 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, |
| 产品应用 | 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 | 146kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Niemann Pick C1 Like 1 |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 储 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 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. |
| 产品介绍 | background: The protein encoded by this gene is a multi-pass membrane protein. It contains a conserved N-terminal Niemann-Pick C1 (NPC1) domain and a putative sterol-sensing domain (SSD) which includes a YQRL motif functioning as a plasma membrane to trans-Golgi network transport signal in other proteins. This protein takes up free cholesterol into cells through vesicular endocytosis and plays a critical role in the absorption of intestinal cholesterol. It also has the ability to transport alpha-tocopherol (vitamin E). The drug ezetimibe targets this protein and inhibits the absorption of intestinal cholesterol and alpha-tocopherol. In addition, this protein may play a critical role in regulating lipid metabolism. Polymorphic variations in this gene are associated with plasma total cholesterol and low-density lipoprotein cholesterol (LDL-C) levels and coronary heart disease (CHD) risk. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Oct 2009] Function: Play a major role in cholesterol homeostasis. Is critical for the uptake of cholesterol across the plasma membrane of the intestinal enterocyte. Is the direct molecular target of ezetimibe, a drug that inhibits cholesterol absorbtion. Lack of activity leads to multiple lipid transport defects. The protein may have a function in the transport of multiple lipids and their homeostasis, and may play a critical role in regulating lipid metabolism. Subcellular Location: Apical cell membrane. Cell membrane. Cytoplasmic vesicle membrane. Subfractionation of brush border membranes from proximal enterocytes suggests considerable association with the apical membrane fraction. Exists as a predominantly cell surface membrane expressed protein (By similarity). According to PubMed:15671032, localizes in a subcellular vesicular compartment rich in RAB5. Tissue Specificity: Widely expressed. Expressed in liver. Also expressed in small intestine, pancreas, kidney, lung, pancreas, spleen, heart, gall bladder, brain, testis, stomach and muscle. Post-translational modifications: Highly glycosylated. Similarity: Belongs to the patched family. Contains 1 SSD (sterol-sensing) domain. Database links: Entrez Gene: 29881 Human Omim: 608010 Human SwissProt: Q9UHC9 Human Unigene: 567486 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
该基因编码的蛋白为多价膜蛋白。它包含一个保守的N端Niemann-Pick C1(NPC1)结构域和一个推测的甾醇感测结构域(SSD)，该结构域包括YQRL基序，其作为质膜在其他蛋白质中跨高尔基体网络传输信号。该蛋白通过囊泡内吞作用将游离胆固醇吸收到细胞内，在肠胆固醇的吸收中起关键作用。它还具有运输α-生育酚（维生素E）的能力。药物依折麦布靶向该蛋白并抑制肠胆固醇和α-生育酚的吸收。此外，该蛋白可能在调节脂质代谢中起关键作用。该基因的多态性与血浆总胆固醇和低密度脂蛋白胆固醇（LDL-C）水平和冠心病（CHD）风险相关。另一种编码不同的异构体的剪接转录变体已被发现用于该基因。[ RefSeq，OCT 2009提供]
