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

Catalog #: MAB10105 Datasheet / COA / SDS

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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Summary Data Examples Preparation and Storage Reconstitution Calculator Background Product Datasheets Related Research Areas

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 either lyophilized or 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.

Scientific Data

Western Blot

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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

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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 Antibody Specificity by Using Knockout Cell Line.

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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

Preparation and Storage

Reconstitution

Reconstitute at 0.5 mg/mL in sterile 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.

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