Rabbit anti-Human Niemann Pick C1/NPC1 Polyclonal Antibody | anti-NPC1 antibody

Anti-Niemann Pick C1/NPC1 Antibody, Rabbit Polyclonal

Cat. Num. AAA8107756

• Gene Names: NPC1; NPC; SLC65A1
• Reactivity: Human
• Applications: ELISA, Immunohistochemistry
• Purity: Protein A & Antigen Affinity
• Synonyms: Niemann Pick C1/NPC1; Polyclonal Antibody; Anti-Niemann Pick C1/NPC1 Antibody; Rabbit Polyclonal; NPC1/Niemann-Pick C1 protein Antibody; Rabbit PAb; Antigen Affinity Purified; Niemann-Pick disease; type C1; Anti-NPC Antibody; anti-NPC1 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: Rabbit IgG
• Specificity: Human NPC1/Niemann-Pick C1 protein
• Purity/Purification: Protein A & Antigen Affinity
• Form/Format: Liquid; 0.2um filtered solution in PBS

• Applicable Applications for anti-NPC1 antibody: ELISA (EIA), Immunohistochemistry-Paraffin (IHC-P)
• Application Notes: ELISA: 1:5000-1:10,000; IHC-P: 1:200-1:500
• Immunogen: Recombinant Human NPC1/Niemann-Pick C1 protein Protein
• Conjugation: Unconjugated
• Preparation: Produced in rabbits immunized with purified, recombinant Human NPC1/Niemann-Pick C1 protein (rh NPC1/Niemann-Pick C1 protein). NPC1/Niemann-Pick C1 protein specific IgG was purified by Human NPC1/Niemann-Pick C1 protein affinity chromatography.
• Preparation and Storage: This antibody can be stored at 2-8 degree C for one month without detectable loss of activity. Antibody products are stable for twelve months from date of receipt when stored at -20 degree C to -80 degree C. Preservative-Free. Avoid repeated freeze-thaw cycles.

Immunohistochemistry (IHC)

(Immunochemical staining of human NPC1 in human kidney with rabbit polyclonal antibody (1:300, formalin-fixed paraffin embedded sections).)

Related Product Information for anti-NPC1 antibody

Niemann-Pick C1 (NPC1), a host receptor involved in the envelope glycoprotein (GP)-mediated entry of filoviruses into cells, is believed to be a major determinant of cell susceptibility to filovirus infection. Niemann-Pick C1 (NPC1), a membrane protein of lysosomes, is required for the export of cholesterol derived from receptor-mediated endocytosis of LDL. The NPC1 protein is a multipass transmembrane protein whose deficiency causes the autosomal recessive lipid storage disorder Niemann-Pick type C1. NPC1 localizes predominantly to late endosomes and has a dileucine motif located within a small cytoplasmic tail thought to target the protein to this location. Niemann-Pick disease type C1 (NPC1) is a rare progressive neurodegenerative disorder caused by mutations in the NPC1 gene. On the cellular level, NPC1 mutations lead to an accumulation of cholesterol and gangliosides.

NCBI and Uniprot Product Information

• NCBI GI #: 255652944
• NCBI GeneID: 4864
• NCBI Accession #: NP_000262.2
• NCBI GenBank Nucleotide #: NM_000271.4
• UniProt Accession #: O15118; Q9P130; B4DET3
• Molecular Weight: 107,025 Da
• NCBI Official Full Name: Niemann-Pick C1 protein
• NCBI Official Synonym Full Names: NPC intracellular cholesterol transporter 1
• NCBI Official Symbol: NPC1
• NCBI Official Synonym Symbols: NPC; SLC65A1
• NCBI Protein Information: Niemann-Pick C1 protein
• UniProt Protein Name: Niemann-Pick C1 protein
• Protein Family: Niemann-Pick C1 protein
• UniProt Gene Name: NPC1
• UniProt Entry Name: NPC1_HUMAN

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]

Uniprot Description

NPC1: Intracellular cholesterol transporter which acts in concert with NPC2 and plays an important role in the egress of cholesterol from the endosomal/lysosomal compartment. Both NPC1 and NPC2 function as the cellular 'tag team duo' (TTD) to catalyze the mobilization of cholesterol within the multivesicular environment of the late endosome (LE) to effect egress through the limiting bilayer of the LE. NPC2 binds unesterified cholesterol that has been released from LDLs in the lumen of the late endosomes/lysosomes and transfers it to the cholesterol-binding pocket of the N-terminal domain of NPC1. Cholesterol binds to NPC1 with the hydroxyl group buried in the binding pocket and is exported from the limiting membrane of late endosomes/ lysosomes to the ER and plasma membrane by an unknown mechanism. 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. Defects in NPC1 are the cause of Niemann-Pick disease type C1 (NPC1). A lysosomal storage disorder that affects the viscera and the central nervous system. It is due to defective intracellular processing and transport of low-density lipoprotein derived cholesterol. It causes accumulation of cholesterol in lysosomes, with delayed induction of cholesterol homeostatic reactions. Niemann-Pick disease type C1 has a highly variable clinical phenotype. Clinical features include variable hepatosplenomegaly and severe progressive neurological dysfunction such as ataxia, dystonia and dementia. The age of onset can vary from infancy to late adulthood. An allelic variant of Niemann-Pick disease type C1 is found in people with Nova Scotia ancestry. Patients with the Nova Scotian clinical variant are less severely affected. Belongs to the patched family.

Protein type: Membrane protein, integral; Membrane protein, multi-pass

Chromosomal Location of Human Ortholog: 18q11.2

Cellular Component: Golgi apparatus; membrane; lysosome; perinuclear region of cytoplasm; integral to plasma membrane; lysosomal membrane; late endosome membrane; endoplasmic reticulum; extracellular region; integral to membrane; nuclear envelope; lipid raft

Molecular Function: protein binding; transmembrane receptor activity; sterol transporter activity; hedgehog receptor activity; cholesterol binding; receptor activity

Biological Process: response to drug; cholesterol metabolic process; lysosomal transport; cholesterol transport; bile acid metabolic process; protein amino acid glycosylation; cholesterol efflux; endocytosis; signal transduction; adult walking behavior; negative regulation of macroautophagy; cholesterol homeostasis; response to cadmium ion; autophagy; lipid raft organization and biogenesis

Disease: Niemann-pick Disease, Type C1