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Niemann-Pick Disease, Type C1 抗体

(Niemann-Pick Disease, Type C1 (NPC1))
This gene encodes a large protein that resides in the limiting membrane of endosomes and lysosomes and mediates intracellular cholesterol trafficking via binding of cholesterol to its N-terminal domain. It is predicted to have a cytoplasmic C-terminus, 13 transmembrane domains, and 3 large loops in the lumen of the endosome - the last loop being at the N-terminus. This protein transports low-density lipoproteins to late endosomal/lysosomal compartments where they are hydrolized and released as free cholesterol. Defects in this gene cause Niemann-Pick type C disease, a rare autosomal recessive neurodegenerative disorder characterized by over accumulation of cholesterol and glycosphingolipids in late endosomal/lysosomal compartments.[provided by RefSeq, Aug 2009].

50 results :

NPC1 适用: 人 ELISA, IHC, FACS 宿主: 小鼠 Monoclonal 8D10G3 unconjugated
Pubmed 2 references
产品编号 ABIN1724840
 
NPC1 适用: 人 ELISA, IHC 宿主: 小鼠 Monoclonal 8D10B6 unconjugated
Pubmed 2 references
产品编号 ABIN1724876
 
NPC1 适用: 人 ELISA, WB, IHC, IF 宿主: 兔 Polyclonal unconjugated
产品编号 ABIN3031998
 
NPC1 适用: 人 ELISA, WB, IHC 宿主: 兔 Polyclonal unconjugated
产品编号 ABIN7161727
 
NPC1 适用: 人 ELISA, WB 宿主: 小鼠 Monoclonal 4H2 unconjugated
产品编号 ABIN562034
 
NPC1 适用: 人 WB, IHC, IF, IP, IHC (p) 宿主: 小鼠 Monoclonal unconjugated
产品编号 ABIN462125
 
NPC1 适用: 人 WB, IF, IHC (p) 宿主: 兔 Polyclonal RB33297 unconjugated
产品编号 ABIN657395
 
Verified NPC1 适用: 人 ELISA, WB 宿主: 山羊 Polyclonal unconjugated
产品编号 ABIN1590132
 
NPC1 适用: 人, 小鼠, Hamster WB, IHC, IF, IP 宿主: 兔 Polyclonal unconjugated
产品编号 ABIN363474
 
NPC1 适用: 人 WB, IHC (p), EIA 宿主: 兔 Polyclonal unconjugated
产品编号 ABIN500383
 
NPC1 适用: 小鼠 ELISA, WB, IHC (p), ICC, IF (cc), IF (p), IHC (fro) 宿主: 兔 Polyclonal unconjugated
产品编号 ABIN872416
 
NPC1 适用: 人 宿主: 兔 Polyclonal FITC
产品编号 ABIN7161729
 
NPC1 适用: 人 ELISA 宿主: 兔 Polyclonal Biotin
产品编号 ABIN7161728
 
NPC1 适用: 人 ELISA 宿主: 兔 Polyclonal HRP
产品编号 ABIN7161730
 
NPC1 适用: 人, 小鼠, 大鼠 ELISA, WB 宿主: 兔 Polyclonal unconjugated
产品编号 ABIN7094638
 
NPC1 适用: 人, 小鼠 ELISA, WB, IF, IHC (p) 宿主: 兔 Polyclonal unconjugated
产品编号 ABIN6991123
 
NPC1 适用: 人 ELISA, WB, IHC 宿主: 兔 Polyclonal unconjugated
产品编号 ABIN7570511
 
NPC1 适用: 人 ELISA, WB, IHC 宿主: 兔 Polyclonal unconjugated
产品编号 ABIN7570510
 
Recent NPC1 适用: 小鼠 ELISA 宿主: 山羊 Polyclonal unconjugated
产品编号 ABIN570865
 
NPC1 适用: 人 ELISA, WB, IHC, IHC (p) 宿主: 小鼠 Monoclonal 8D10B6 unconjugated
产品编号 ABIN1918106
 
  • 类型 Primary
    • Primary
  • 应用范围
    • ELISA
    • Western Blotting (WB)
    • Immunohistochemistry (IHC)
    • Immunofluorescence (IF)
    • Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))
    • Immunocytochemistry (ICC)
    • Immunoprecipitation (IP)
    • Flow Cytometry (FACS)
    • Enzyme Immunoassay (EIA)
    • Immunofluorescence (Cultured Cells) (IF (cc))
    • Immunofluorescence (Paraffin-embedded Sections) (IF (p))
    • Immunohistochemistry (Frozen Sections) (IHC (fro))
  • 适用
    • Human
    • Mouse
    • Rat
    • Hamster
  • 论文引用
  • 图像
  • Preservative free only
  • Carrier free only
  • 抗体来源
    • Rabbit
    • Mouse
    • Goat
  • 克隆形成能力
    • Polyclonal
    • Monoclonal
  • 克隆
    • 8D10B6
    • 18N07
    • 4H2
    • 8D10G3
    • RB33297
  • 抗原表位
    • AA 23-269
    • Internal Region
    • AA 591-620
    • AA 590-620
    • Center
    • AA 34-174
    • C-Term
    • AA 1022-1278
    • AA 1228-1278
    • AA 116-620
    • AA 1181-1278
    • AA 151-250
    • AA 23-266
    • AA 372-622
    • Middle Region
    • N-Term
  • 标记
    • 非结合性
    • FITC
    • Biotin
    • APC
    • Alexa Fluor 488
    • Alexa Fluor 555
    • Alexa Fluor 594
    • Alexa Fluor 647
    • Alexa Fluor 680
    • Alexa Fluor 750
    • HRP
    • PE
  • 抗体亚型
    • IgG
    • IgG1
    • Ig Fraction
    • IgG2a
  • 蛋白类型
    • Recombinant Antibody
  • Format
    • Liquid
    • Lyophilized
  • Grade
    • Recent
    • Verified
  • Supplier
    • antibodies-online
    • Signalway
    • Boster Bio
    • USBio
    • FabGennix
    • NSJ Bioreagents
    • Abbexa
    • Abcepta
    • Acris
    • Biomatik
    • Covalab

Latest Publications for our Niemann-Pick Disease, Type C1 抗体

Ferrante, De Nuccio, Pepponi, Visentin, Martire, Bernardo, Minghetti, Popoli: "Stimulation of adenosine A2A receptors reduces intracellular cholesterol accumulation and rescues mitochondrial abnormalities in human neural cell models of Niemann-Pick C1." in: Neuropharmacology, Vol. 103, pp. 155-62, (2016) (PubMed).

Ebrahimi-Fakhari, Wahlster, Bartz, Werenbeck-Ueding, Praggastis, Zhang, Joggerst-Thomalla, Theiss, Grimm, Ory, Runz: "Reduction of TMEM97 increases NPC1 protein levels and restores cholesterol trafficking in Niemann-pick type C1 disease cells." in: Human molecular genetics, (2016) (PubMed).

Li, Deffieu, Lee, Saha, Pfeffer: "Glycosylation inhibition reduces cholesterol accumulation in NPC1 protein-deficient cells." in: Proceedings of the National Academy of Sciences of the United States of America, Vol. 112, Issue 48, pp. 14876-81, (2015) (PubMed).

Maetzel, Sarkar, Wang, Abi-Mosleh, Xu, Cheng, Gao, Mitalipova, Jaenisch: "Genetic and chemical correction of cholesterol accumulation and impaired autophagy in hepatic and neural cells derived from Niemann-Pick Type C patient-specific iPS cells." in: Stem cell reports, Vol. 2, Issue 6, pp. 866-80, (2014) (PubMed).

Armstrong, Mattsson, Appelqvist, Janefjord, Sandin, Agholme, Olsson, Svensson, Blennow, Zetterberg, Kågedal: "Lysosomal network proteins as potential novel CSF biomarkers for Alzheimer's disease." in: Neuromolecular medicine, Vol. 16, Issue 1, pp. 150-60, (2014) (PubMed).

Zampieri, Bembi, Rosso, Filocamo, Dardis: "Treatment of Human Fibroblasts Carrying NPC1 Missense Mutations with MG132 Leads to an Improvement of Intracellular Cholesterol Trafficking." in: JIMD reports, Vol. 2, pp. 59-69, (2013) (PubMed).

Ohgane, Karaki, Dodo, Hashimoto: "Discovery of oxysterol-derived pharmacological chaperones for NPC1: implication for the existence of second sterol-binding site." in: Chemistry & biology, Vol. 20, Issue 3, pp. 391-402, (2013) (PubMed).

Fu, Wassif, Yanjanin, Watkins-Chow, Baxter, Incao, Liscum, Sidhu, Firnkes, Graham, Ory, Porter, Pavan: "Efficacy of N-acetylcysteine in phenotypic suppression of mouse models of Niemann-Pick disease, type C1." in: Human molecular genetics, Vol. 22, Issue 17, pp. 3508-23, (2013) (PubMed).

Roszell, Tao, Yu, Gao, Huang, Ning, Feinstein, Vite, Bates: "Pulmonary abnormalities in animal models due to Niemann-Pick type C1 (NPC1) or C2 (NPC2) disease." in: PLoS ONE, Vol. 8, Issue 7, pp. e67084, (2013) (PubMed).

Cianciola, Greene, Morton, Carlin: "Adenovirus RIDα uncovers a novel pathway requiring ORP1L for lipid droplet formation independent of NPC1." in: Molecular biology of the cell, Vol. 24, Issue 21, pp. 3309-25, (2013) (PubMed).

Aliases for Niemann-Pick Disease, Type C1 抗体

NPC intracellular cholesterol transporter 1 (NPC1) 抗体
NPC1 like intracellular cholesterol transporter 1 (Npc1l1) 抗体
NPC intracellular cholesterol transporter 1 (Npc1) 抗体
Niemann-Pick disease, type C1 (npc1) 抗体
Niemann-Pick C1 protein (LOC579887) 抗体
9130221N23Rik 抗体
A430089E03Rik 抗体
C85354 抗体
Cdig2 抗体
D18Ertd139e 抗体
D18Ertd723e 抗体
Gm243 抗体
im:7149020 抗体
lcsd 抗体
nmf164 抗体
NPC 抗体
spm 抗体
wu:fb53a12 抗体
wu:fc29a12 抗体
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