Human/Mouse/Rat Niemann-Pick Type C1/NPC1 Alexa Fluor® 405-conjugated Antibody
R&D Systems, part of Bio-Techne | Catalog # FAB10105V
Recombinant Monoclonal Antibody.
Conjugate
Catalog #
Key Product Details
Species Reactivity
Human, Mouse, Rat
Applications
Knockout Validated, Immunohistochemistry, Western Blot
Label
Alexa Fluor 405 (Excitation = 405 nm, Emission = 421 nm)
Antibody Source
Recombinant Monoclonal Rabbit IgG1 Clone # 1318A
Product Specifications
Immunogen
Synthetic peptide containing Human Niemann-Pick Type C1/NPC1
Specificity
Detects human Niemann-Pick Type C1/NPC1 in direct ELISAs. Detects human, mouse, and rat Niemann-Pick Type C1/NPC1 in Western blots.
Clonality
Monoclonal
Host
Rabbit
Isotype
IgG1
Applications
Application
Recommended Usage
Immunohistochemistry
Optimal dilution of this antibody should be experimentally determined.
Knockout Validated
Optimal dilution of this antibody should be experimentally determined.
Western Blot
Optimal dilution of this antibody should be experimentally determined.
Formulation, Preparation, and Storage
Purification
Protein A or G purified
Formulation
Supplied 0.2mg/ml in 1X PBS with RDF1 and 0.09% Sodium Azide
Shipping
The product is shipped with polar packs. Upon receipt, store it immediately at the temperature recommended below.
Stability & Storage
Protect from light. Do not freeze. 12 months from date of receipt, 2 to 8 °C as supplied
Background: Niemann-Pick Type C1/NPC1
NPC intracellular cholesterol transporter 1 or Niemann-Pick C1 protein (NPC1) is a 1,278 aminoacids (aa) intracellular cholesterol transporter which plays an important role in cholesterol transport from the endosomal/lysosomal compartment. NPC1 was identified as the gene that when mutated, results in Niemann-Pick disease, type C, a rare neurovisceral lipid storage disorder resulting from autosomal recessively inherited loss-of-function mutations in either NPC1 or NPC2. This disrupts intracellular lipid transport, leading to the accumulation of lipid products in the late endosomes and lysosomes. Approximately 95% of NPC patients are found to have mutations in the NPC1 gene. In humans, at least one other isoform, missing aa 519-586 is known. Human NPC1 protein sequence is 87% identical to both, mouse and rat NPC1.
Additional Niemann-Pick Type C1/NPC1 Products
Product Specific Notices
This product is provided under an agreement between Life Technologies Corporation and R&D Systems, Inc, and the manufacture, use, sale or import of this product is subject to one or more US patents and corresponding non-US equivalents, owned by Life Technologies Corporation and its affiliates. The purchase of this product conveys to the buyer the non-transferable right to use the purchased amount of the product and components of the product only in research conducted by the buyer (whether the buyer is an academic or for-profit entity). The sale of this product is expressly conditioned on the buyer not using the product or its components (1) in manufacturing; (2) to provide a service, information, or data to an unaffiliated third party for payment; (3) for therapeutic, diagnostic or prophylactic purposes; (4) to resell, sell, or otherwise transfer this product or its components to any third party, or for any other commercial purpose. Life Technologies Corporation will not assert a claim against the buyer of the infringement of the above patents based on the manufacture, use or sale of a commercial product developed in research by the buyer in which this product or its components was employed, provided that neither this product nor any of its components was used in the manufacture of such product. For information on purchasing a license to this product for purposes other than research, contact Life Technologies Corporation, Cell Analysis Business Unit, Business Development, 29851 Willow Creek Road, Eugene, OR 97402, Tel: (541) 465-8300. Fax: (541) 335-0354.
For research use only
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