Niemann-Pick Disease, Type C1 Produits
(Niemann-Pick Disease, Type C1 (NPC1))
Catégories
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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Catégories Niemann-Pick Disease, Type C1 en vedette
Niemann-Pick Disease, Type C1 Anticorps
High quality antibodies with extensive validation data.
Niemann-Pick Disease, Type C1 Kits ELISA
Reliable ELISA kits for a wide range of species.
Niemann-Pick Disease, Type C1 Protéines
Proteins for various applications incl. WB, ELISA, IF etc.
Niemann-Pick Disease, Type C1 recommandé Anticorps
Produit
Reactivity
Application
Validations
N° du produit
Quantité
Fiche technique
Reactivity
Human
Application
ELISA, WB, IHC, FACS
Validations
- (2)
- (6)
N° du produit
ABIN1724840
Quantité
100 μL
Fiche technique
Fiche technique
Reactivity
Human
Application
ELISA, WB, IHC
Validations
- (2)
- (5)
N° du produit
ABIN1724876
Quantité
100 μL
Fiche technique
Fiche technique
Reactivity
Human
Application
ELISA, WB, IHC, IF
Validations
- (5)
N° du produit
ABIN3031998
Quantité
0.4 mL
Fiche technique
Fiche technique
Niemann-Pick Disease, Type C1 recommandé Kits ELISA
Produit
Reactivity
Analytical Method
Validations
N° du produit
Quantité
Fiche technique
Reactivity
Human
Analytical Method
Quantitative Sandwich ELISA
Validations
- (1)
N° du produit
ABIN6958195
Quantité
96 tests
Fiche technique
Fiche technique
Reactivity
Human
Analytical Method
Quantitative Sandwich ELISA
Validations
N° du produit
ABIN6951470
Quantité
96 tests
Fiche technique
Fiche technique
Reactivity
Chicken
Analytical Method
Quantitative
Validations
N° du produit
ABIN1056422
Quantité
96 tests
Fiche technique
Fiche technique
Niemann-Pick Disease, Type C1 recommandé Protéines
Produit
Reactivity
Source
Validations
N° du produit
Quantité
Fiche technique
Reactivity
Human
Source
Wheat germ
Validations
- (1)
N° du produit
ABIN1312849
Quantité
10 μg
Fiche technique
Fiche technique
Reactivity
Human
Source
HEK-293 Cells
Validations
- (1)
N° du produit
ABIN7317151
Quantité
100 μg
Fiche technique
Fiche technique
Reactivity
Human, Mouse, Rat
Source
Escherichia coli (E. coli)
Validations
N° du produit
ABIN2750647
Quantité
5 applications
Fiche technique
Fiche technique
Dernières publications sur nos produits Niemann-Pick Disease, Type C1
: "Stimulation of adenosine A2A receptors reduces intracellular cholesterol accumulation and rescues mitochondrial abnormalities in human neural cell models of Niemann-Pick C1." dans: 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." dans: Human molecular genetics, (2016) (PubMed).
: "Glycosylation inhibition reduces cholesterol accumulation in NPC1 protein-deficient cells." dans: 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." dans: Stem cell reports, Vol. 2, Issue 6, pp. 866-80, (2014) (PubMed).
: "Lysosomal network proteins as potential novel CSF biomarkers for Alzheimer's disease." dans: 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." dans: 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." dans: 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." dans: 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." dans: 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." dans: Molecular biology of the cell, Vol. 24, Issue 21, pp. 3309-25, (2013) (PubMed).
Synonymes et noms alternatifs relatifs à Niemann-Pick Disease, Type C1
NPC intracellular cholesterol transporter 1 (NPC1), NPC1 like intracellular cholesterol transporter 1 (Npc1l1), NPC intracellular cholesterol transporter 1 (Npc1), Niemann-Pick disease, type C1 (npc1), Niemann-Pick C1 protein (LOC579887), 9130221N23Rik, A430089E03Rik, C85354, Cdig2, D18Ertd139e, D18Ertd723e, Gm243, im:7149020, lcsd, nmf164, NPC, spm, wu:fb53a12, wu:fc29a12Protein level used designations for Niemann-Pick Disease, Type C1
- Niemann-Pick C1 protein
- Niemann-Pick C1-like protein 1
- Niemann-Pick disease, type C1
- Niemann-Pick disease, type C1-like 1
- Niemann-Pick type C1 disease protein
- Nasopharyngeal carcinoma 1
- Niemann-Pick C1
- sphingomyelinosis
- Niemann-Pick C disease protein
- Niemann Pick type C1
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