NPC1 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].
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Popular NPC1 抗体
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Reactivity
Application
Validations
Cat. No.
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Datasheet
Reactivity
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Application
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Cat. No.
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Quantity
0.1 mg
Datasheet
Datasheet
Reactivity
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Application
IF, IHC, WB
Validations
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Cat. No.
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Quantity
100 μL
Datasheet
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Cat. No.
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Cat. No.
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Cat. No.
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Quantity
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Datasheet
Datasheet
Reactivity
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Application
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Cat. No.
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Quantity
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Datasheet
Reactivity
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Application
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Cat. No.
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Application
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Cat. No.
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Datasheet
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Cat. No.
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Cat. No.
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Quantity
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Cat. No.
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Quantity
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Datasheet
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Reactivity
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Cat. No.
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Quantity
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Datasheet
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Reactivity
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Cat. No.
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Datasheet
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Reactivity
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Datasheet
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Latest Publications for our NPC1 抗体
: "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).: "Reduction of TMEM97 increases NPC1 protein levels and restores cholesterol trafficking in Niemann-pick type C1 disease cells." in: Human molecular genetics, (2016) (PubMed).
: "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).
: "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).
: "Lysosomal network proteins as potential novel CSF biomarkers for Alzheimer's disease." in: Neuromolecular medicine, Vol. 16, Issue 1, pp. 150-60, (2014) (PubMed).
: "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).
: "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).
: "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).
: "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).
: "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 NPC1 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
NPC intracellular cholesterol transporter 1 (Npc1) 抗体
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