Human/Mouse/Rat Niemann-Pick Type C1/NPC1 Antibody
R&D Systems, part of Bio-Techne | Catalog # MAB10105
Recombinant Monoclonal Antibody.
Key Product Details
Validated by
Knockout/Knockdown
Species Reactivity
Human, Mouse, Rat
Applications
Immunohistochemistry, Knockout Validated, Western Blot
Label
Unconjugated
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
Scientific Data Images for Human/Mouse/Rat Niemann-Pick Type C1/NPC1 Antibody
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.
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.
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.Applications for Human/Mouse/Rat Niemann-Pick Type C1/NPC1 Antibody
Application
Recommended Usage
Immunohistochemistry
3-25 µg/mL
Sample: Immersion fixed paraffin-embedded sections of human brain (cerebellum)
Sample: Immersion fixed paraffin-embedded sections of human brain (cerebellum)
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.
Western Blot
0.5 µg/mL
Sample: A172 human glioblastoma cell line, Neuro‑2A mouse neuroblastoma cell line, and PC‑12 rat adrenal pheochromocytoma cell line
Sample: A172 human glioblastoma cell line, Neuro‑2A mouse neuroblastoma cell line, and PC‑12 rat adrenal pheochromocytoma cell line
Formulation, Preparation, and Storage
Purification
Protein A or G purified from cell culture supernatant
Reconstitution
Reconstitute at 0.5 mg/mL in sterile PBS. For liquid material, refer to CoA for concentration.
Formulation
Lyophilized from a 0.2 μm filtered solution in PBS with Trehalose. *Small pack size (SP) is supplied either lyophilized or as a 0.2 µm filtered solution in PBS.
Shipping
Lyophilized product is shipped at ambient temperature. Liquid small pack size (-SP) is shipped with polar packs. Upon receipt, store immediately at the temperature recommended below.
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.
Additional Niemann-Pick Type C1/NPC1 Products
Product Documents for Human/Mouse/Rat Niemann-Pick Type C1/NPC1 Antibody
Product Specific Notices for Human/Mouse/Rat Niemann-Pick Type C1/NPC1 Antibody
For research use only
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