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RabbitAnti-NPC2 Rabbit pAb  antibody (bs-11736R)
~~~促销,代码KXJ230206~~~
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说明书: 50ul  100ul  200ul
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价

产品编号 bs-11736R
英文名称 NPC2 Rabbit pAb
中文名称 尼曼匹克C2前体蛋白抗体
别    名 EDDM1; Niemann Pick C2; 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.  
研究领域 肿瘤  心血管  细胞生物  神经生物学  信号转导  
抗体来源 Rabbit
克隆类型 Polyclonal
克 隆 号
交叉反应 Mouse,  (predicted: Human, Rat, Cow, Sheep, )
产品应用 WB=1:500-2000 ELISA=1:5000-10000 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 15 kDa
检测分子量
细胞定位 细胞浆 分泌型蛋白 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Niemann Pick C2: 20-80/151 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20℃ for one year. 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 This gene encodes a protein containing a lipid recognition domain. The encoded protein may function in regulating the transport of cholesterol through the late endosomal/lysosomal system. Mutations in this gene have been associated with Niemann-Pick disease, type C2 and frontal lobe atrophy. [provided by RefSeq, Jul 2008]

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.

SWISS:
P61916

Gene ID:
10577

Database links:

Entrez Gene: 10577 Human

Omim: 601015 Human

SwissProt: P61916 Human

SwissProt: P61918 Monkey

Unigene: 433222 Human



产品图片
Sample: Lane 1: Recombinant human NPC2 protein, N-His(bs-42193P) Primary: Anti-NPC2 (bs-11736R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 15 kDa Observed band size: 15 kDa
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