antibody

NPC1 Antibody

MBS150200

0.1 mg

345 USD

polyclonal

rabbit

nonconjugated

western blot, ELISA, immunohistochemistry, immunocytochemistry, enzyme immunoassay

Antibody

NPC1 Antibody

NPC1

NPC1; NPC; Niemann-Pick C1 protein; Niemann-Pick disease, type C1

Niemann-Pick C1 protein; Niemann-Pick C1 protein; Niemann-Pick C1 protein; Niemann-Pick disease, type C1

NPC1

NPC1; NPC1; NPC

NPC1_HUMAN

Polyclonal

IgG

Rabbit

Human

1278

NPC1 Antibody is affinity chromatography purified via peptide column.

Liquid

1 mg/mL

NPC1 antibody can be stored at 4 degree C for three months and -20 degree C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.

ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC), Immunofluorescence (IF)

NPC1 antibody can be used for detection of NPC1 by Western blot at 1 mug/mL. Antibody can also be used for immunohistochemistry starting at 2.5 mug/mL. For immunofluorescence start at 20 mug/mL.

Conjugate: Unconjugated. Immunogen: NPC1 antibody was raised against a 16 amino acid synthetic peptide from near the carboxy terminus of human NPC1. Buffer: NPC1 Antibody is supplied in PBS containing 0.02% sodium azide.

NPC1 Antibody: Mutations in the Niemann-Pick disease type C1 (NPC1) gene result in a fatal progressive neurodegenerative disorder characterized by an abnormal sequestration of lipids including cholesterol and glycosphingolipids. The NPC1 protein is 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. NPC1 transports low-density lipoproteins to late endosomal/lysosomal compartments where they are hydrolized and released as free cholesterol. NPC1, in addition to FTO, MC4R, and PTER has recently been shown to be a new risk loci for early-onset and morbid adult obesity in European populations. This anti-NPC1 antibody will not cross-react to NPC2, another gene whose defects also result in Niemann-Pick type C disease.

255652944

NP_000262

NM_000271.4

O15118

107,025 Da

Lysosome Pathway 99052!!Lysosome Pathway 96865

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]

0.1 mg

345 USD