| 产品编号 | bsm-62437R |
| 英文名称 | NPC1 Recombinant Rabbit mAb |
| 中文名称 | 尼曼匹克C1前体蛋白重组兔单抗 |
| 别 名 | NPC; POGZ; SLC65A1; A430089E03Rik; D18Ertd139e; D18Ertd723e; lcsd; nmf164; spm; Cdig2; SLC66A1; NPC1_HUMAN; NPC1; Niemann-Pick C1 protein; NPC1_MOUSE; |
| 抗体来源 | Rabbit |
| 克隆类型 | Recombinant |
| 克 隆 号 | 9F4 |
| 交叉反应 | Human,Mouse,Rat |
| 产品应用 | IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,Flow-Cyt=1:50-100,ICC/IF=1:50-200
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理论分子量 | 142 kDa |
| 检测分子量 | 180 |
| 性 状 | Liquid |
| 免 疫 原 | A synthesized peptide derived from human Niemann pick C1: 1240-1278 |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 缓 冲 液 | 10mM phosphate buffered saline(pH 7.4) with 150mM sodium chloride, 0.05% BSA, 0.02% Proclin300 and 50% glycerol. |
| 保存条件 | Store at 4℃ for short term. Store at -20℃ for long term. Avoid repeated freeze/thaw cycles. |
| 注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 产品介绍 |
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-神经磷沉积性疾病，主要是由于神经磷脂酶（sphingomyelinase）缺乏所致，神经鞘磷脂酶（sphingomyelinase）缺乏致神经鞘磷脂代谢障碍。导致后者蓄积在单核巨噬细胞系统内，出现肝、脾肿大，中枢神经系统退行性变。神经鞘磷脂是由N-酰鞘氨醇与一个分子的磷酸胆硷（phosphocholine）在C1、部位连接而成，神经鞘磷脂来源于各种细胞膜和红细胞基质等。在细胞代谢衰老过程中被巨噬细胞吞噬，神经磷脂酶缺少后，全身神经鞘磷脂代谢紊乱，神经磷脂沉积在单核-巨噬细胞系统和神经组织细胞中 |
| 1、抗体溶解方法 | |
| 2、抗体修复方式 | |
| 3、常用试剂的配制 | |
| 4、免疫组化操作步骤 | |
| 5、免疫组化问题解答 | |
| 6、Western Blotting 操作步骤 | |
| 7、Western Blotting 问题解答 | |
| 8、关于肽链的设计 | |
| 9、多肽的溶解与保存 | |
| 10、酶标抗体效价测定程序 | |
