Niemann-Pick Disease, Type C1 (NPC1) Antikörper

Antigen: Niemann-Pick Disease, Type C1 (NPC1)

Synonyme: NPC, FLJ98532, Gm243, 9130221N23Rik, RP23-198N14.2, Npc1, NPC1, im:7149020, wu:fb53a12, wu:fc29a12

Klonalität: Polyklonal

Wirt: Kaninchen

Reaktivität: Human, Maus

Applikation: Immunhistochemie (Gefrierschnitte) (IHC (fro)), Immunfluoreszenz (IF), Immunpräzipitation (IP), Western Blot (WB)

Produktnummer: ABIN268885

Produktbeschreibung

Weitere Bezeichnung: NPC1

Gen-ID: 4864

Immunogen: A synthetic peptide made to the C-terminal region of human NPC1.

Beschreibung: Niemann-Pick type C1 (NPC1) is a member of a family of genes encodingmembrane-bound proteins containing putative sterol sensing domains. NPC1 proteinregulates cholesterol transport from late endosomes-lysosomes to other intracellularcompartments. NPC1 overexpression increases the rate of trafficking of low densitylipoprotein cholesterol to the endoplasmic reticulum and the rate of delivery ofendosomal cholesterol to the plasma membrane. NPC disease is an inherited neurovisceral lipid storage disorder of unesterifiedcholesterol accumulation in lysosomes. It is characterized by progressive neural andliver degeneration, resulting from inactivating mutations in NPC1, in most cases. Alternate Names: anti-NPC antibody, anti-NPC1 antibody, anti-Niemann Pick C1 antibody, anti-NiemannPick C1 protein precursor antibody, anti-Niemann Pick disease antibody, anti-NPCantibody, anti-NPC1 antibody, anti-Niemann Pick C1 antibody, anti-Niemann Pick C1protein precursor antibody, anti-Niemann Pick disease antibody.

Spezifität: This antibody is specific for NPC1 protein. Species Reactivity: This antibody reacts with human, mouse, and hamster protein. Other species have notbeen tested. Localization: Late endosome and Lysosome membrane, Single-pass membrane protein.

Anwendungen

Applikationshinweise: Suggested working dilutions: immunofluorescence 1:250,Western Blot 1:1000-1:3000 (ECL). Positive Controls: Human fibroblast cell lysate

Konzentration: 1.3 mg/ml

Buffer: PBS. Preservative: 0.025% sodium azide.

Lagerung: Store at 4C short term. Aliquot and store at -20C long term. Avoid freeze thaw cycles.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Publikationen

Publikationen: Millard, Srivastava, Traub et al.: "Niemann-pick type C1 (NPC1) overexpression alters cellular cholesterol homeostasis." in: The Journal of biological chemistry, Vol. 275, Issue 49, pp. 38445-51, 2001 (PubMed).

Infante, Abi-Mosleh, Radhakrishnan et al.: "Purified NPC1 protein. I. Binding of cholesterol and oxysterols to a 1278-amino acid membrane protein." in: The Journal of biological chemistry, Vol. 283, Issue 2, pp. 1052-63, 2008 (PubMed).

Burke, Colvin, Myatt et al.: "Transport of maternal cholesterol to the fetus is affected by maternal plasma cholesterol concentrations in the Golden Syrian hamster." in: Journal of lipid research, Vol. 50, Issue 6, pp. 1146-55, 2009 (PubMed).

Podechard, Le Ferrec, Rebillard et al.: "NPC1 repression contributes to lipid accumulation in human macrophages exposed to environmental aryl hydrocarbons." in: Cardiovascular research, Vol. 82, Issue 2, pp. 361-70, 2009 (PubMed).