尼曼匹克C2前体蛋白抗体品牌 英文名称  Anti-NPC2/Niemann Pick C2

中文名称  尼曼匹克C2前体蛋白抗体品牌 

别    名 EDDM1; Epididymal protein 1; Epididymal secretory protein; Epididymal secretory protein E1; HE1; Human epididymis-specific protein 1; Niemann-Pick disease type C2; Niemann-Pick disease type C2 protein; NPC2; NPC2_HUMAN; Tissue specific secretory protein.

产品属性：

浓    度  1mg/1ml

规 格  0.2ml/200μg

抗体来源  Rabbit

克隆类型   polyclonal

交叉反应   Human, Mouse, Rat, Cow, Sheep

产品类型   一抗  

研究领域     心 细胞生物 神经生物学 信号转导

蛋白分子量  predicted molecular weight: 15kDa 

性    状   Lyophilized or Liquid

免 疫 原  KLH conjugated synthetic peptide derived from human Niemann Pick C2 (20-80aa)

亚    型  IgG

纯化方法   affinity purified by Protein A

储 存 液   Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4

尼曼匹克C2前体蛋白抗体品牌 产品应用   WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500

（石蜡切片需做抗原修复） 

 not yet tested in other applications.

 optimal dilutions/concentrations should be determined by the end user.  

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. 

Important Note  This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 

产品介绍 NPC2 is a secreted protein mapping against gene 14q24.3 (1,2). NPC2 regulates the lipid composition of sperm membranes during maturation in the epididymis(1,2). Mutations in the NPC2 gene may cause Nieman-Pick type C2 disease and frontal lobe atrophy (1,2,3). Nieman-Pick type C2 is a fatal hereditary disease characterized by defective lysosome release of cholesterol (3). The disease is caused by HE1 deficiency, a lysosmal protein proven to be undetectable in fibroblasts from NPC2 patients (3). This differentiates NPC2 from NPC1, as NPC1 has HE1 protein present (3).

Function : May be involved in the regulation of the lipid composition of sperm membranes during the maturation in the epididymis.Subunit : Interacts with NUS1/NgBR, the interaction stabilizes NCP2 and regulates cholesterol trafficking. Interacts with DHDDS. Interacts with NPC1 (via the second lumenal domain) in a cholestrol-dependent manner (By similarity). Interacts with NEDD4L (via C2 domain) (By similarity). Interacts with NPC1L1.Subcellular Location : Secreted. Endoplasmic reticulum. Lysosome.Tissue Specificity : Epididymis.DISEASE : Defects in NPC2 are the cause of Niemann-Pick disease type C2 (NPDC2) [MIM:607625]. A lysosomal storage disorder that affects the viscera and the central nervous system. It is due to defective intracellular processing and transport of low-density lipoprotein derived cholesterol. It causes accumulation of cholesterol in lysosomes, with delayed induction of cholesterol homeostatic reactions. Niemann-Pick disease type C2 has a highly variable clinical phenotype. Clinical features include variable hepatosplenomegaly and severe progressive neurological dysfunction such as ataxia, dystonia and dementia. The age of onset can vary from infancy to late adulthood.Similarity : Belongs to the NPC2 family.Database links : UniProtKB/Swiss-Prot: P61916.1

实验流程：

全、新、优、品、好四大特点：

尼曼匹克C2前体蛋白抗体品牌 全：公司提供上万种产品，涵盖了生物试剂，elisa试剂盒，标准品，培养基，原装耗材，抗体、培养基、ATCC细胞等，基本上各种科研所需产品在我司都能找到。

新：产品更新速度较快，基本上每周都有新产品出现。

优：产品质量好，投诉比较少。

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