Human/Mouse/Rat Niemann-Pick Type C1/NPC1 Antibody

Recombinant Monoclonal Antibody
Catalog # Availability Size / Price Qty
MAB10105-100
MAB10105-SP
Detection  of Human, Mouse, and Rat Niemann-Pick Type C1/NPC1 by Western Blot.
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Human/Mouse/Rat Niemann-Pick Type C1/NPC1 Antibody Summary

Species Reactivity
Human, Mouse, Rat
Specificity
Detects human Niemann-Pick Type C1/NPC1 in direct ELISAs. Detects human, mouse, and rat Niemann-Pick Type C1/NPC1 in Western blots.
Source
Recombinant Monoclonal Rabbit IgG1 Clone # 1318A
Purification
Protein A or G purified from cell culture supernatant
Immunogen
Synthetic peptide containing Human Niemann-Pick Type C1/NPC1
Formulation
Lyophilized from a 0.2 μm filtered solution in PBS with Trehalose. *Small pack size (SP) is supplied as a 0.2 µm filtered solution in PBS.
Label
Unconjugated

Applications

Recommended Concentration
Sample
Western Blot
0.5 µg/mL
See below
Immunohistochemistry
3-25 µg/mL
See below
Knockout Validated
Niemann-Pick Type C1/NPC1 is specifically detected in HeLa human cervical epithelial carcinoma parental cell line but is not detectable in Niemann-Pick Type C1/NPC1 knockout HeLa cell line.
 

Please Note: Optimal dilutions should be determined by each laboratory for each application. General Protocols are available in the Technical Information section on our website.

Data Examples

Western Blot Detection  of Human, Mouse, and Rat Niemann-Pick Type C1/NPC1 by Western Blot. View Larger

Detection of Human, Mouse, and Rat Niemann-Pick Type C1/NPC1 by Western Blot. Western blot shows lysates of A172 human glioblastoma cell line, Neuro‑2A mouse neuroblastoma cell line, and PC‑12 rat adrenal pheochromocytoma cell line. PVDF membrane was probed with 0.5 µg/mL of Rabbit Anti-Human/Mouse/Rat Niemann-Pick Type C1/NPC1 Monoclonal Antibody (Catalog # MAB10105) followed by HRP-conjugated Anti-Rabbit IgG Secondary Antibody (Catalog # HAF008). A specific band was detected for Niemann-Pick Type C1/NPC1 at approximately 170-250 kDa (as indicated). This experiment was conducted under reducing conditions and using Immunoblot Buffer Group 1.

Immunohistochemistry Niemann-Pick  Type C1/NPC1 in Human Brain. View Larger

Niemann-Pick Type C1/NPC1 in Human Brain. Niemann-Pick Type C1/NPC1 was detected in immersion fixed paraffin-embedded sections of human brain (cerebellum) using Rabbit Anti-Human/Mouse/Rat Niemann-Pick Type C1/NPC1 Monoclonal Antibody (Catalog # MAB10105) at 3 µg/mL for 1 hour at room temperature followed by incubation with the Anti-Rabbit IgG VisUCyte™ HRP Polymer Antibody (Catalog # VC003). Before incubation with the primary antibody, tissue was subjected to heat-induced epitope retrieval using Antigen Retrieval Reagent-Basic (Catalog # CTS013). Tissue was stained using DAB (brown) and counterstained with hematoxylin (blue). Specific staining was localized to synaptic puncta. View our protocol for IHC Staining with VisUCyte HRP Polymer Detection Reagents.

Knockout Validated Western  Blot Shows Human Niemann-Pick Type C1/NPC1 Specificity by Using Knockout Cell  Line. View Larger

Western Blot Shows Human Niemann-Pick Type C1/NPC1 Specificity by Using Knockout Cell Line. Western blot shows lysates of HeLa human cervical epithelial carcinoma parental cell line and Niemann-Pick Type C1/NPC1 knockout HeLa cell line (KO). PVDF membrane was probed with 0.5 µg/mL of Rabbit Anti-Human/Mouse/Rat Niemann-Pick Type C1/NPC1 Monoclonal Antibody (Catalog # MAB10105) followed by HRP-conjugated Anti-Rabbit IgG Secondary Antibody (Catalog # HAF008). A specific band was detected for Niemann-Pick Type C1/NPC1 at approximately 170-250 kDa (as indicated) in the parental HeLa cell line, but is not detectable in knockout HeLa cell line. GAPDH (Catalog # MAB5718) is shown as a loading control. This experiment was conducted under reducing conditions and using Immunoblot Buffer Group 1.

Reconstitution Calculator

Reconstitution Calculator

The reconstitution calculator allows you to quickly calculate the volume of a reagent to reconstitute your vial. Simply enter the mass of reagent and the target concentration and the calculator will determine the rest.

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Preparation and Storage

Reconstitution
Reconstitute at 0.5 mg/mL in sterile PBS.
Reconstitution Buffer Available
Reconstitution Buffer 1 (PBS)
Catalog #
Availability
Size / Price
Qty
RB01
Shipping
The product is shipped at ambient temperature. Upon receipt, store it immediately at the temperature recommended below. *Small pack size (SP) is shipped with polar packs. Upon receipt, store it immediately at -20 to -70 °C
Stability & Storage
Use a manual defrost freezer and avoid repeated freeze-thaw cycles.
  • 12 months from date of receipt, -20 to -70 °C as supplied.
  • 1 month, 2 to 8 °C under sterile conditions after reconstitution.
  • 6 months, -20 to -70 °C under sterile conditions after reconstitution.

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.

Entrez Gene IDs
4864 (Human); 18145 (Mouse)
Alternate Names
FLJ98532; Niemann-Pick C1 protein; Niemann-Pick disease, type C1; Niemann-Pick Type C1; NPC; NPC1; SLC65A1

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