Niemann-Pick Disease, Type C1 Produkte
(Niemann-Pick Disease, Type C1 (NPC1))
Kategorien
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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Ausgewählte Niemann-Pick Disease, Type C1 Kategorien
Niemann-Pick Disease, Type C1 Antikörper
High quality antibodies with extensive validation data.
Niemann-Pick Disease, Type C1 ELISA Kits
Reliable ELISA kits for a wide range of species.
Niemann-Pick Disease, Type C1 Proteine
Proteins for various applications incl. WB, ELISA, IF etc.
Empfohlene Niemann-Pick Disease, Type C1 Antikörper
Produkt
Reaktivität
Applikation
Validierungen
Kat. Nr.
Menge
Datenblatt
Reaktivität
Human
Applikation
ELISA, WB, IHC, FACS
Validierungen
- (2)
- (6)
Kat. Nr.
ABIN1724840
Menge
100 μL
Datenblatt
Datenblatt
Reaktivität
Human
Applikation
ELISA, WB, IHC
Validierungen
- (2)
- (5)
Kat. Nr.
ABIN1724876
Menge
100 μL
Datenblatt
Datenblatt
Reaktivität
Human
Applikation
ELISA, WB, IHC, IF
Validierungen
- (5)
Kat. Nr.
ABIN3031998
Menge
0.4 mL
Datenblatt
Datenblatt
Empfohlene Niemann-Pick Disease, Type C1 ELISA Kits
Produkt
Reaktivität
Analytical Method
Validierungen
Kat. Nr.
Menge
Datenblatt
Reaktivität
Human
Analytical Method
Quantitative Sandwich ELISA
Validierungen
- (1)
Kat. Nr.
ABIN6958195
Menge
96 tests
Datenblatt
Datenblatt
Reaktivität
Human
Analytical Method
Quantitative Sandwich ELISA
Validierungen
Kat. Nr.
ABIN6951470
Menge
96 tests
Datenblatt
Datenblatt
Reaktivität
Chicken
Analytical Method
Quantitative
Validierungen
Kat. Nr.
ABIN1056422
Menge
96 tests
Datenblatt
Datenblatt
Empfohlene Niemann-Pick Disease, Type C1 Proteine
Produkt
Reaktivität
Source
Validierungen
Kat. Nr.
Menge
Datenblatt
Reaktivität
Human
Source
Wheat germ
Validierungen
- (1)
Kat. Nr.
ABIN1312849
Menge
10 μg
Datenblatt
Datenblatt
Reaktivität
Human
Source
HEK-293 Cells
Validierungen
- (1)
Kat. Nr.
ABIN7317151
Menge
100 μg
Datenblatt
Datenblatt
Reaktivität
Human, Mouse, Rat
Source
Escherichia coli (E. coli)
Validierungen
Kat. Nr.
ABIN2750647
Menge
5 applications
Datenblatt
Datenblatt
Neueste Publikationen zu unseren Niemann-Pick Disease, Type C1 Produkten
: "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).
Synonyme und alternative Namen zu 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:fc29a12Bezeichner auf Proteinebene für 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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