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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].
NPC1 抗体  (AA 34-174) NPC1 抗体  (AA 34-174) NPC1 抗体 (AA 34-174) (ABIN1724840)

NPC1 适用: 人 ELISA, WB, IHC, FACS 宿主: 小鼠 Monoclonal 8D10G3 unconjugated

NPC1 抗体  (AA 34-174) NPC1 抗体  (AA 34-174) NPC1 抗体 (AA 34-174) (ABIN1724876)

NPC1 适用: 人 ELISA, WB, IHC 宿主: 小鼠 Monoclonal 8D10B6 unconjugated

NPC1 抗体  (AA 34-174) NPC1 抗体  (AA 34-174) NPC1 抗体 (AA 34-174) (ABIN1845969)

NPC1 适用: 人 ELISA, WB, IHC, FACS 宿主: 小鼠 Monoclonal unconjugated

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Popular Niemann-Pick Disease, Type C1 抗体

Product
Reactivity
Application
Validations
Cat. No.
Quantity
Datasheet
Reactivity Human
Application ELISA, WB, IHC, FACS
Validations
  • (2)
  • (6)
Cat. No. ABIN1724840
Quantity 0.1 mg
Datasheet Datasheet
Reactivity Human
Application ELISA, WB, IHC
Validations
  • (2)
  • (5)
Cat. No. ABIN1724876
Quantity 0.1 mg
Datasheet Datasheet
Reactivity Human
Application ELISA, WB, IHC, FACS
Validations
  • (6)
Cat. No. ABIN1845969
Quantity 100 μL
Datasheet Datasheet
Reactivity Human
Application ELISA, WB, IHC, IF
Validations
  • (5)
Cat. No. ABIN3031998
Quantity 0.4 mL
Datasheet Datasheet
Reactivity Human
Application ELISA, WB, IHC
Validations
  • (3)
Cat. No. ABIN7161727
Quantity 100 μg
Datasheet Datasheet
Reactivity Human
Application ELISA, WB
Validations
  • (3)
Cat. No. ABIN562034
Quantity 100 μg
Datasheet Datasheet
Reactivity Human
Application WB, IHC, IF, IHC (p), IP
Validations
  • (3)
Cat. No. ABIN462125
Quantity 50 μg
Datasheet Datasheet
Reactivity Hamster, Human, Mouse
Application WB, IHC, IF, IP, IEM
Validations
  • (3)
  • (1)
Cat. No. ABIN540364
Quantity 100 μL
Datasheet Datasheet
Reactivity Human
Application WB, IF, IHC (p)
Validations
  • (3)
Cat. No. ABIN657395
Quantity 400 μL
Datasheet Datasheet
Reactivity Human
Application ELISA, WB
Validations
  • (1)
Cat. No. ABIN1590132
Quantity 100 μg
Datasheet Datasheet
Reactivity Human
Application WB, IHC (p), EIA
Validations
  • (2)
Cat. No. ABIN500383
Quantity 0.1 mg
Datasheet Datasheet
Reactivity Hamster, Human, Mouse
Application WB, IHC, IF, IP
Validations
  • (2)
Cat. No. ABIN363474
Quantity 0.1 mL
Datasheet Datasheet
Reactivity Mouse
Application ELISA, WB, IHC (p), ICC, IF (cc), IF (p), IHC (fro)
Validations
Cat. No. ABIN872416
Quantity 100 μL
Datasheet Datasheet
Reactivity Human, Mouse, Rat
Application WB, IHC (p), FACS, IF (cc), IF (p)
Validations
Cat. No. ABIN6944307
Quantity 100 μL
Datasheet Datasheet
Reactivity Human
Application ELISA
Validations
Cat. No. ABIN7161728
Quantity 100 μg
Datasheet Datasheet

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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