| 產品編號 | bsm-62437R |
| 英文名稱 | NPC1 Recombinant Rabbit mAb |
| 中文名稱 | 尼曼匹克C1前體蛋白重組兔單抗 |
| 別 名 | Niemann Pick C1 protein precursor; NPC; NPC1. |
| 抗體來源 | Rabbit |
| 克隆類型 | Recombinant |
| 克 隆 號 | |
| 交叉反應 | Human,Mouse,Rat |
| 產品應用 | WB=1:1000-1:2000,IHC-P=1:100-1:200,IHC-F=1:100-1:200,IF=1:50-1:200,Flow-Cyt=1:20-1:100,ICC/IF=1:50-1:200
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 142 kDa |
| 檢測分子量 | 180 |
| 性 狀 | Liquid |
| 免 疫 原 | A synthesized peptide derived from human Niemann pick C1: 1240-1278 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 緩 沖 液 | 10mM phosphate buffered saline(pH 7.4) with 150mM sodium chloride, 0.05% BSA, 0.02% Proclin300 and 50% glycerol. |
| 保存條件 | Store at 4℃ for short term. Store at -20℃ for long term. Avoid repeated freeze/thaw cycles. |
| 注意事項 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 產品介紹 |
Intracellular cholesterol transporter which acts in concert with NPC2 and plays an important role in the egress of cholesterol from the endosomal/lysosomal compartment.Unesterified cholesterol that has been released from LDLs in the lumen of the late endosomes/lysosomes is transferred by NPC2 to the cholesterol-binding pocket in the N-terminal domain of NPC1. SWISS: O15118 Gene ID: 4864 尼曼匹克病-神經磷沉積性疾病，主要是由于神經磷脂酶（sphingomyelinase）缺乏所致，神經鞘磷脂酶（sphingomyelinase）缺乏致神經鞘磷脂代謝障礙。導致后者蓄積在單核巨噬細胞系統(tǒng)內，出現肝、脾腫大，中樞神經系統(tǒng)退行性變。神經鞘磷脂是由N-酰鞘氨醇與一個分子的磷酸膽鹼（phosphocholine）在C1、部位連接而成，神經鞘磷脂來源于各種細胞膜和紅細胞基質等。在細胞代謝衰老過程中被巨噬細胞吞噬，神經磷脂酶缺少后，全身神經鞘磷脂代謝紊亂，神經磷脂沉積在單核-巨噬細胞系統(tǒng)和神經組織細胞中 |
| 產品圖片 |
Western blot analysis of HepG2 cell lysate. Using NPC1 (bsm-62437R) monoclonal antibody at 1:1000 dilution and 4°C overnight incubation. Followed by conjugated secondary antibody incubation at r.t. for 60 min.
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| 1、抗體溶解方法 | |
| 2、抗體修復方式 | |
| 3、常用試劑的配制 | |
| 4、免疫組化操作步驟 | |
| 5、免疫組化問題解答 | |
| 6、Western Blotting 操作步驟 | |
| 7、Western Blotting 問題解答 | |
| 8、關于肽鏈的設計 | |
| 9、多肽的溶解與保存 | |
| 10、酶標抗體效價測定程序 | |
