Rabbit Polyclonal Niemann Pick C2 antibody. Suitable for IHC-P, WB and reacts with Human samples. Immunogen corresponding to Recombinant Fragment Protein within Human NPC2 aa 1-150.
pH: 7
Preservative: 0.01% Thimerosal (merthiolate)
Constituents: 20% Glycerol (glycerin, glycerine), 1.21% Tris, 0.75% Glycine
IHC-P | WB | |
---|---|---|
Human | Tested | Tested |
Species | Dilution info | Notes |
---|---|---|
Species Human | Dilution info 1/100 - 1/500 | Notes Perform heat-mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol. |
Species | Dilution info | Notes |
---|---|---|
Species Human | Dilution info 1/500 - 1/3000 | Notes - |
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Intracellular cholesterol transporter which acts in concert with NPC1 and plays an important role in the egress of cholesterol from the lysosomal compartment (PubMed:11125141, PubMed:15937921, PubMed:17018531, PubMed:18772377, PubMed:29580834). 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:17018531, PubMed:18772377, PubMed:27238017). May bind and mobilize cholesterol that is associated with membranes (PubMed:18823126). NPC2 binds cholesterol with a 1:1 stoichiometry (PubMed:17018531). Can bind a variety of sterols, including lathosterol, desmosterol and the plant sterols stigmasterol and beta-sitosterol (PubMed:17018531). The secreted form of NCP2 regulates biliary cholesterol secretion via stimulation of ABCG5/ABCG8-mediated cholesterol transport (By similarity).
HE1, NPC2, NPC intracellular cholesterol transporter 2, Epididymal secretory protein E1, Human epididymis-specific protein 1, Niemann-Pick disease type C2 protein, He1
Rabbit Polyclonal Niemann Pick C2 antibody. Suitable for IHC-P, WB and reacts with Human samples. Immunogen corresponding to Recombinant Fragment Protein within Human NPC2 aa 1-150.
pH: 7
Preservative: 0.01% Thimerosal (merthiolate)
Constituents: 20% Glycerol (glycerin, glycerine), 1.21% Tris, 0.75% Glycine
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The Niemann Pick C2 protein (NPC2) is a small intracellular cholesterol transport protein weighing approximately 16 kDa. Alternately referred to as "NPC2 protein" or "NPC 293" it binds with lipids and is predominantly expressed in the lysosome. This protein plays a critical role in the intracellular lipid transport process. NPC2 forms part of a family of proteins tasked with moving cholesterol and other related lipids within lysosomal compartments.
NPC2 protein facilitates the transfer of cholesterol from lysosomes to other parts of the cell. It operates in tandem with its partner protein Niemann Pick C1 (NPC1) to mediate the egress of cholesterol. The NPC2 protein docks to NPC1 within the lysosomal membrane forming an essential complex in lipid transport. This function is pivotal for the maintenance of cellular cholesterol homeostasis influencing various cellular and systemic functions linked to cholesterol metabolism.
NPC2 protein participates prominently in the cholesterol efflux pathways. It associates with the NPC1 protein to regulate the movement of cholesterol from the lysosomal membrane to cytoplasmic areas where it can be utilized or stored. This pathway links NPC2 to significant biological processes like the homeostasis of lipoprotein-derived cholesterol influencing the SREBP pathway which governs lipid synthesis and uptake. Such interactions highlight NPC2's involvement in regulating cellular lipid balance.
NPC2 protein dysfunction notably links to Niemann-Pick disease type C a lipid storage disorder. Mutations in the genes encoding NPC2 or NPC1 disrupt cholesterol transportation leading to abnormal lipid accumulation and progressive neurodegeneration. Additionally this protein's dysregulation is indirectly connected to atherosclerosis as such disruptions can result in altered cellular cholesterol management highlighting its interaction with proteins involved in cardiovascular diseases.
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ab126190, at 1/500 dilution, staining Niemann Pick C2 in Paraffin-embedded Human U87 xenograft tissue by Immunohistochemistry.
15% SDS PAGE
All lanes: Western blot - Anti-Niemann Pick C2 antibody (ab126190) at 1/1000 dilution
All lanes: HeLa whole cell lysate at 30 µg
Predicted band size: 17 kDa, 29 kDa
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