Western Blot (WB)
(Western Blot: Sample: Recombinant protein.)
Rabbit anti-Human Niemann Pick Disease Type C2 Polyclonal Antibody | anti-NPC2 antibody
Niemann Pick Disease Type C2 (NPC2) Polyclonal Antibody
Immunohistochemistry in formalin fixed frozen section: 1:50-500
Enzyme-linked Immunosorbent Assay: 1:100-200
Usage: 10uL per well when 3,3'-Diaminobenzidine(DAB) as the substrate.
5uL per well when used in enhanced chemilumescent (ECL).
Note: The quality control is specifically manufactured as the positive control.Not used for other purposes.
Loading Buffer: 100mM Tris(pH8.8), 2% SDS, 200mM NaCl, 50% glycerol,BPB 0.01%, NaN3 0.02%.
Western Blot (WB)
(Western Blot: Sample: Recombinant protein.)
Western Blot (WB)
(Western Blot: Sample: Recombinant protein.)
Immunohistochemistry (IHC)
(DABstainingonIHC-P.Samples:HumanTissue))
Immunohistochemistry (IHC)
(DABstainingonIHC-P.Samples:HumanTissue))
NCBI and Uniprot Product Information
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: endoplasmic reticulum; extracellular region; lysosomal lumen; lysosome
Molecular Function: cholesterol binding; cholesterol transporter activity; enzyme binding; protein binding
Biological Process: cholesterol efflux; cholesterol homeostasis; cholesterol metabolic process; cholesterol transport; glycolipid transport; intracellular cholesterol transport; intracellular sterol transport; neutrophil degranulation; phospholipid transport; regulation of isoprenoid metabolic process; response to virus
Disease: Niemann-pick Disease, Type C2
Research Articles on NPC2
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Product Notes
The NPC2 npc2 (Catalog #AAA2032486) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The Niemann Pick Disease Type C2 (NPC2) Polyclonal Antibody reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's Niemann Pick Disease Type C2 can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), Immunocytochemistry (ICC), Immunohistochemistry (IHC) Formalin/Paraffin, ELISA (EIA). Western Blot: 1:50-400 Immunohistochemistry in formalin fixed frozen section: 1:50-500 Enzyme-linked Immunosorbent Assay: 1:100-200. Researchers should empirically determine the suitability of the NPC2 npc2 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "Niemann Pick Disease Type C2, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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