Anti-NPC1 Antibody

About this product

Boster Bio Anti-NPC1 Antibody (Catalog # A00428) . Tested in ELISA, WB, IHC-P, IF applications. This antibody reacts with Human, Mouse.

Specifications

Catalog number

A00428

Size / count

0.1 mg

Dry ice

No

Hazardous

No

Discontinued

No

Background

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.

Synonyms

NPC, Niemann-Pick C1 protein

Gene Name

NPC1

UniProt

O15118

Host

Rabbit

Reactivity

Human, Mouse

Immunogen

NPC1 antibody was raised against a 16 amino acid synthetic peptide from near the carboxy terminus of human NPC1. The immunogen is located within the last 50 amino acids of NPC1.

Clonality

Polyclonal

Applications

ELISA, WB, IHC-P, IF

Field of Research

Obesity

Purification

NPC1 Antibody is affinity chromatography purified via peptide column.

Concentration

1 mg/mL

Form

Liquid

Function

Intracellular cholesterol transporter which acts in concert with NPC2 and plays an important role in the egress of cholesterol from the endosomal/lysosomal compartment (PubMed:9211849, PubMed:9927649, PubMed:10821832, PubMed:18772377, PubMed:27238017, PubMed:12554680) . Unesterified cholesterol that has been released from LDLs in the lumen of the late endosomes/lysosomes is transferred by NPC2 to the cholesterol- binding pocket in the N-terminal domain of NPC1 (PubMed:9211849, PubMed:9927649, PubMed:18772377, PubMed:19563754, PubMed:27238017, PubMed:28784760) . Cholesterol binds to NPC1 with the hydroxyl group buried in the binding pocket (PubMed:19563754) . Binds oxysterol with higher affinity than cholesterol. May play a role in vesicular trafficking in glia, a process that may be crucial for maintaining the structural and functional integrity of nerve terminals (Probable) .

Storage Conditions

NPC1 antibody can be stored at 4°C for three months and -20°C, stable for up to one year. Avoid repeated freeze-thaw cycles. Antibodies should not be exposed to prolonged high temperatures.

Observed Molecular Weight

142167 MW

Gene Name Synonym

Niemann-Pick disease, type C1

Subcellular Location

Late endosome membrane

Prediction Reactivity

Pig

Protein Name

Niemann-Pick disease, type C1

Isotype

IgG

Contents

NPC1 Antibody is supplied in PBS containing 0.02% sodium azide.