Rabbit NPC2 Polyclonal Antibody | anti-NPC2 antibody

NPC2 Antibody

Cat. Num. AAA9406120

• Gene Names: NPC2; HE1; EDDM1
• Reactivity: Human, Mouse, Rat
• Applications: Western Blot, Immunohistochemistry, Immunofluorescence
• Purity: Antibodies were purified by affinity purification using immunogen.
• Synonyms: NPC2; Polyclonal Antibody; NPC2 Antibody; HE1; EDDM1; anti-NPC2 antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Specificity: The antibody detects endogenous level of total NPC2 protein.
• Purity/Purification: Antibodies were purified by affinity purification using immunogen.
• Form/Format: Supplied at 1.0mg/mL in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
• Concentration: 1.0 mg/ml (varies by lot)
• Sequence Length: 151

• Applicable Applications for anti-NPC2 antibody: Western Blot (WB), Immunohistochemistry (IHC), Immunofluorescence (IF)
• Application Notes: Western blotting: 1:500 - 1:2000; Immunohistochemistry: 1:50 - 1:200; Immunofluorescence: 1:50 - 1:200
• Immunogen Type: Recombinant Protein
• Immunogen Description: Recombinant protein of human NPC2.
• Target Name: NPC2
• Preparation and Storage: Store at -20 degree C

Western Blot (WB)

(Western blot analysis of extracts of various cell lines, using NPC2 antibody.)

Immunohistochemistry (IHC)

(Immunohistochemical analysis of paraffin-embedded human kidney using NPC2 antibody at dilution of 1:200 (400x lens).)

Immunohistochemistry (IHC)

(Immunohistochemical analysis of paraffin-embedded human liver cancer using NPC2 antibody at dilution of 1:200 (200x lens).)

Immunohistochemistry (IHC)

(Immunohistochemical analysis of paraffin-embedded human lung using NPC2 antibody at dilution of 1:200 (400x lens).)

Immunofluorescence (IF)

(Immunofluorescence analysis of U20S cell using NPC2 antibody. Blue: DAPI for nuclear staining.)

Related Product Information for anti-NPC2 antibody

This gene encodes a protein containing a lipid recognition domain. The encoded protein may function in regulating the transport of cholesterol through the late endosomal/lysosomal system. Mutations in this gene have been associated with Niemann-Pick disease, type C2 and frontal lobe atrophy.

Product Categories/Family for anti-NPC2 antibody

Total protein Ab

NCBI and Uniprot Product Information

• NCBI GI #: 5453678
• NCBI GeneID: 10577
• NCBI Accession #: NP_006423.1
• NCBI GenBank Nucleotide #: NM_006432.3
• UniProt Accession #: P61916; Q15668; Q29413; B4DQV7
• Molecular Weight: 13,416 Da
• NCBI Official Full Name: epididymal secretory protein E1
• NCBI Official Synonym Full Names: Niemann-Pick disease, type C2
• NCBI Official Symbol: NPC2
• NCBI Official Synonym Symbols: HE1; EDDM1
• NCBI Protein Information: epididymal secretory protein E1
• UniProt Protein Name: Epididymal secretory protein E1
• Protein Family: Non-specific phospholipase
• UniProt Gene Name: NPC2
• UniProt Synonym Gene Names: HE1; He1
• UniProt Entry Name: NPC2_HUMAN

NCBI Description

This gene encodes a protein containing a lipid recognition domain. The encoded protein may function in regulating the transport of cholesterol through the late endosomal/lysosomal system. Mutations in this gene have been associated with Niemann-Pick disease, type C2 and frontal lobe atrophy. [provided by RefSeq, Jul 2008]

Uniprot Description

NPC2: Intracellular cholesterol transporter which acts in concert with NPC1 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. The secreted form of NCP2 regulates biliary cholesterol secretion via stimulation of ABCG5/ABCG8-mediated cholesterol transport. Defects in NPC2 are the cause of Niemann-Pick disease type C2 (NPDC2). 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 C2 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. Belongs to the NPC2 family.

Protein type: Secreted; Secreted, signal peptide

Chromosomal Location of Human Ortholog: 14q24.3

Cellular Component: lysosome; endoplasmic reticulum

Molecular Function: protein binding; enzyme binding; cholesterol binding

Biological Process: cholesterol metabolic process; cholesterol homeostasis; cholesterol transport; intracellular sterol transport; regulation of isoprenoid metabolic process; response to virus; cholesterol efflux; phospholipid transport; intracellular cholesterol transport; glycolipid transport

Disease: Niemann-pick Disease, Type C2