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  • 产品名称：Niemann-PickDisease,TypeC2(NPC2)(AA20-151)(Active)protein(Histag)

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  • 产品厂商：ACROBiosystems
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简单介绍：
Niemann-PickDisease,TypeC2(NPC2)(AA20-151)(Active)protein(Histag)
详情介绍：
Characteristics This protein carries a polyhistidine tag at the C-terminus. The protein has a calculated MW of 16.5 kDa. The protein migrates as 20-24 kDa under reducing (R) condition (SDS-PAGE) due to glycosylation.
Purity >95 % as determined by SDS-PAGE.
Sterility 0.22 μm filtered
Endotoxin Level Less than 1.0 EU per μg by the LAL method.
Background Niemann-Pick disease type C2 protein (NPC2) is also known as epididymal secretory protein E1 and human epididymis-specific protein 1 (HE1). NPC2 is a intracellular cholesterol transporter which acts in concert with NPC1 and plays an important role in the egress of cholesterol from the endosomal/lysosomal compartment. Both NPC1 and NPC2 function as the cellular 'tag team duo' (TTD) to catalyze the mobilization of cholesterol within the multivesicular environment of the late endosome (LE) to effect egress through the limiting bilayer of the LE. Also,NPC2 binds unesterified cholesterol that has been released from LDLs in the lumen of the late endosomes/lysosomes and transfers it to the cholesterol-binding pocket of the N-terminal domain of NPC1. Furthermore,the secreted form of NCP2 regulates biliary cholesterol secretion via stimulation of ABCG5/ABCG8-mediated cholesterol transport.
Molecular Weight 16.5 kDa
UniProt P61916
Restrictions For Research Use only
Format Lyophilized
Reconstitution Please see Certificate of Analysis for specific instructions. For best performance, we strongly recommend you to follow the reconstitution protocol provided in the CoA.
Buffer PBS, pH 7.4
Handling Advice Avoid repeated freeze-thaw cycles.
Storage -20 °C
Storage Comment No activity loss was observed after storage at: In lyophilized state for 1 year (4 °C), After reconstitution under sterile conditions for 3 months (-70 °C).
Supplier Images
SDS-PAGE (SDS) image for Niemann-Pick Disease, Type C2 (NPC2) (AA 20-151) (Active) protein (His tag) (ABIN2181547) Human Niemann-Pick Type C2, His Tag on SDS-PAGE under reducing (R) condition. The gel...
Background publications Yamanashi, Takada, Shoda, Suzuki: "Novel function of Niemann-Pick C1-like 1 as a negative regulator of Niemann-Pick C2 protein." in: Hepatology (Baltimore, Md.), Vol. 55, Issue 3, pp. 953-64, 2012 (PubMed).

Xu, Farver, Kodukula, Storch: "Regulation of sterol transport between membranes and NPC2." in: Biochemistry, Vol. 47, Issue 42, pp. 11134-43, 2008 (PubMed).

Chen, Yeh, Liou, Kao: "Insulin attenuates endotoxin-induced acute lung injury in conscious rats." in: Critical care medicine, Vol. 34, Issue 3, pp. 758-64, 2006 (PubMed).

Liou, Dixit, Xu, Tint, Stock, Lobel: "NPC2, the protein deficient in Niemann-Pick C2 disease, consists of multiple glycoforms that bind a variety of sterols." in: The Journal of biological chemistry, Vol. 281, Issue 48, pp. 36710-23, 2006 (PubMed).

Morisot, Millat, Coeslier, Bourgois, Fontenoy, Dobbelaere, Verot, Haouari, Vaillant, Gottrand, Bogaert, Thelliez, Klosowski, Djebara, Bachiri, Manouvrier, Vanier: "[Fatal neonatal respiratory distress in Niemann-Pick C2 and prenatal diagnosis with mutations in gene HE1/NPC2]." in: Archives de pediatrie : organe officiel de la Societe francaise de pediatrie, Vol. 12, Issue 4, pp. 434-7, 2005 (PubMed).

Millat, Baïlo, Molinero, Rodriguez, Chikh, Vanier: "Niemann-Pick C disease: use of denaturing high performance liquid chromatography for the detection of NPC1 and NPC2 genetic variations and impact on management of patients and families." in: Molecular genetics and metabolism, Vol. 86, Issue 1-2, pp. 220-32, 2005 (PubMed).