| 產(chǎn)品編號(hào) | 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(lái)源 | Rabbit |
| 克隆類型 | Recombinant |
| 克 隆 號(hào) | |
| 交叉反應(yīng) | Human,Mouse,Rat |
| 產(chǎn)品應(yīng)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(cè)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(xiàng) | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 產(chǎn)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(jīng)磷沉積性疾病，主要是由于神經(jīng)磷脂酶（sphingomyelinase）缺乏所致，神經(jīng)鞘磷脂酶（sphingomyelinase）缺乏致神經(jīng)鞘磷脂代謝障礙。導(dǎo)致后者蓄積在單核巨噬細(xì)胞系統(tǒng)內(nèi)，出現(xiàn)肝、脾腫大，中樞神經(jīng)系統(tǒng)退行性變。神經(jīng)鞘磷脂是由N-酰鞘氨醇與一個(gè)分子的磷酸膽鹼（phosphocholine）在C1、部位連接而成，神經(jīng)鞘磷脂來(lái)源于各種細(xì)胞膜和紅細(xì)胞基質(zhì)等。在細(xì)胞代謝衰老過(guò)程中被巨噬細(xì)胞吞噬，神經(jīng)磷脂酶缺少后，全身神經(jīng)鞘磷脂代謝紊亂，神經(jīng)磷脂沉積在單核-巨噬細(xì)胞系統(tǒng)和神經(jīng)組織細(xì)胞中 |
| 產(chǎn)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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