Human/Mouse/Rat Niemann-Pick Type C1/NPC1 Alexa Fluor™ Plus 594-conjugated Antibody
R&D Systems, part of Bio-Techne | Catalog # FAB10105AFP594
Conjugate
Catalog #
Key Product Details
Species Reactivity
Human, Mouse, Rat
Applications
Knockout Validated, Immunohistochemistry, Western Blot
Label
Alexa Fluor Plus 594 (Excitation = 590 nm, Emission = 618 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
Formulation
Supplied 0.2 mg/mL in a saline solution containing BSA and 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 intellectual property license from Life Technologies Corporation. The transfer of this product is conditioned on the buyer using the purchased product solely in research conducted by the buyer, excluding contract research or any fee for service research, and the buyer must not (1) use this product or its components for (a) diagnostic, therapeutic or prophylactic purposes; (b) testing, analysis or screening services, or information in return for compensation on a per-test basis; or (c) manufacturing or quality assurance or quality control, and/or (2) sell or transfer this product or its components for resale, whether or not resold for use in research. For information on purchasing a license to this product for purposes other than as described above, contact Life Technologies Corporation, 5781 Van Allen Way, Carlsbad, CA 92008 USA or outlicensing@thermofisher.com.
For research use only
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