Mouse anti-Human NPC1 Monoclonal Antibody | anti-NPC1 antibody

NPC1 (Niemann-Pick C1 Protein) (Biotin)

Cat. Num. AAA6143232

• Gene Names: NPC1; NPC; POGZ; SLC65A1
• Reactivity: Human
• Applications: ELISA, Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: NPC1; Monoclonal Antibody; NPC1 (Niemann-Pick C1 Protein) (Biotin); anti-NPC1 antibody

• Host: Mouse
• Reactivity: Human
• Clonality: Monoclonal
• Isotype: IgG2a,k
• Clone Number: 4H2
• Specificity: Recognizes human NPC1.
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Biotin.
• Sequence Length: 4650

• Applicable Applications for anti-NPC1 antibody: ELISA (EIA), Western Blot (WB)
• Application Notes: Applications are based on unconjugated antibody.
• Immunogen: Partial recombinant corresponding to aa151-250 from human NPC1 (AAH63302) with GST tag. MW of the GST tag alone is 26kD.
• Immunogen Sequence: GFANAMYNACRDVEAPSSNDKALGLLCGKDADACNATNWIEYMFNKDNGQAPFTITPVFSDFPVHGMEPMNNATKGCDESVDEVTAPCSCQDCSIVCGPK
• Conjugate: Biotin
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(Western Blot detection against Immunogen (36.63kD).)

Testing Data

(Detection limit for recombinant GST tagged NPC1 is ~0.03ng/ml as a capture antibody.)

Related Product Information for anti-NPC1 antibody

Niemann-Pick C1 (NPC1) disease is characterized by cholesterol accumulation in lysosomes and aberrant feedback regulation of cellular cholesterol homeostasis. The gene responsible for the disease is NPC1, which encodes a protein with five transmembrane domains. NPC1 protein has homology with the resistance-nodulation-division (RND) family of prokaryotic permeases and may normally function as a transmembrane efflux pump. NPC1 uses a proton motive force to remove accumulated acriflavine from the endosomal/lysosomal (E/L) system. NPC1 can function to transport lipophilic molecules but not cholesterol, out of the E/L system. The fact that NPC1 can transport acriflavine and fatty acids suggest that this permease may have a "multidrug" transport function, part of which is its housekeeping role in cellular cholesterol homeostasis. Expression of NPC1 in E. coli facilitated the transport of acriflavine across the plasma membrane, causing cytosolic accumulation and resulting in transport of oleic acid, but not cholesterol-oleate across the plasma membrane, establishing NPC1 as a eukaryotic member of the RND permease family.

Product Categories/Family for anti-NPC1 antibody

Antibodies; Disease Markers

NCBI and Uniprot Product Information

• NCBI GI #: 38649259
• NCBI GeneID: 4864
• NCBI Official Full Name: Homo sapiens Niemann-Pick disease, type C1, mRNA
• NCBI Official Synonym Full Names: NPC intracellular cholesterol transporter 1
• NCBI Official Symbol: NPC1
• NCBI Official Synonym Symbols: NPC; POGZ; SLC65A1
• NCBI Protein Information: NPC intracellular cholesterol transporter 1
• Protein Family: Niemann-Pick C1 protein

NCBI Description

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]