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Rabbit Recombinant Monoclonal Niemann Pick C1 antibody. Suitable for IHC-P, WB, ICC/IF, Flow Cyt (Intra) and reacts with Mouse, Rat, Human samples. Cited in 51 publications.


Images

Key facts

Isotype

IgG

Host species

Rabbit

Storage buffer

pH: 7.2 - 7.4
Preservative: 0.01% Sodium azide
Constituents: 59% PBS, 40% Glycerol (glycerin, glycerine), 0.05% BSA

Form

Liquid

Clonality

Monoclonal

Immunogen

  • The exact immunogen used to generate this antibody is proprietary information.
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Reactivity data

Select an application
Product promiseTestedExpectedPredictedNot recommended
IHC-PWBICC/IFFlow Cyt (Intra)
Human
Tested
Tested
Expected
Expected
Mouse
Tested
Tested
Tested
Tested
Rat
Tested
Expected
Expected
Expected

Tested
Tested

Species

Mouse

Dilution info

1/50 - 1/100

Notes

Perform heat-mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.

Species

Rat

Dilution info

1/50 - 1/100

Notes

Perform heat-mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.

Species

Human

Dilution info

1/50 - 1/100

Notes

Perform heat-mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.

Tested
Tested

Species

Mouse

Dilution info

1/2000 - 1/10000

Notes

-

Species

Human

Dilution info

1/2000 - 1/10000

Notes

-

Expected
Expected

Species

Rat

Dilution info

Use at an assay dependent concentration.

Notes

-

Tested
Tested

Species

Mouse

Dilution info

1/70

Notes

-

Expected
Expected

Species

Rat, Human

Dilution info

Use at an assay dependent concentration.

Notes

-

Tested
Tested

Species

Mouse

Dilution info

1/200

Notes

ab172730 - Rabbit monoclonal IgG, is suitable for use as an isotype control with this antibody.

Expected
Expected

Species

Rat, Human

Dilution info

Use at an assay dependent concentration.

Notes

-

Target data

Function

Intracellular cholesterol transporter which acts in concert with NPC2 and plays an important role in the egress of cholesterol from the endosomal/lysosomal compartment (PubMed:9211849, PubMed:9927649, PubMed:10821832, PubMed:18772377, PubMed:27238017, PubMed:12554680). Unesterified cholesterol that has been released from LDLs in the lumen of the late endosomes/lysosomes is transferred by NPC2 to the cholesterol-binding pocket in the N-terminal domain of NPC1 (PubMed:9211849, PubMed:9927649, PubMed:18772377, PubMed:19563754, PubMed:27238017, PubMed:28784760). Cholesterol binds to NPC1 with the hydroxyl group buried in the binding pocket (PubMed:19563754). Binds oxysterol with higher affinity than cholesterol. May play a role in vesicular trafficking in glia, a process that may be crucial for maintaining the structural and functional integrity of nerve terminals (Probable).(Microbial infection) Acts as an endosomal entry receptor for ebolavirus.

Alternative names

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Rabbit Recombinant Monoclonal Niemann Pick C1 antibody. Suitable for IHC-P, WB, ICC/IF, Flow Cyt (Intra) and reacts with Mouse, Rat, Human samples. Cited in 51 publications.

Alternative names

Key facts

Isotype

IgG

Form

Liquid

Clonality

Monoclonal

Immunogen
  • The exact immunogen used to generate this antibody is proprietary information.
Clone number

EPR5209

Purification technique

Affinity purification Protein A

Dissociation constant

4.9 x 10-11 M

Concentration
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Storage

Shipped at conditions

Blue Ice

Appropriate long-term storage conditions

-20°C

Storage information

Stable for 12 months at -20°C

Notes

Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMAb® patents.

This product is a recombinant monoclonal antibody, which offers several advantages including:

  • - High batch-to-batch consistency and reproducibility
  • - Improved sensitivity and specificity
  • - Long-term security of supply
  • - Animal-free batch production

For more information, read more on recombinant antibodies.

Supplementary info

Biological function summary

This protein acts in the movement of lipids and cholesterol through cellular compartments. NPC1 functions as part of a protein complex that includes NPC2 which works to mobilize cholesterol from lysosomes to other areas in the cell where it is further processed or stored. This transport mechanism is necessary to maintain cellular lipid homeostasis and proper cellular functions.

Activity summary

Niemann Pick C1 also known as NPC1 or C1 protein is an integral membrane protein involved in cholesterol and lipid transport within cells. It is located in late endosomes and lysosomes and plays a role in intracellular cholesterol traffic. The NPC1 protein has a molecular weight of approximately 170 kDa. It is expressed in various tissues with high expression levels in the liver brain and spleen.

Pathways

NPC1 integrates into the cholesterol trafficking pathway and plays an essential part in intracellular lipid regulation. It interacts with proteins involved in cholesterol metabolism such as sterol regulatory element-binding proteins (SREBPs). These interactions ensure that cholesterol distribution within the cell remains balanced and adjusts to metabolic needs. The proper function of NPC1 in these pathways is necessary for maintaining cell health and homeostasis.

Associated diseases and disorders

NPC1 mutations are directly linked to Niemann-Pick disease type C a severe lipid storage disorder. This condition is characterized by the accumulation of cholesterol and other lipids in lysosomes leading to neurological and hepatic dysfunction. NPC1's role in this disease involves disrupted cholesterol trafficking which is critical for cellular lipid homeostasis. Additionally NPC2 protein is also affected in this disorder as both work together in the cholesterol transport process.

Product promise

We are dedicated to supporting your work with high quality reagents and we are here for you every step of the way should you need us.

In the unlikely event of one of our products not working as expected, you are covered by our product promise.

Full details and terms and conditions can be found here:
Terms & Conditions.

13 product images

  • Immunocytochemistry/ Immunofluorescence - Anti-Niemann Pick C1 antibody [EPR5209] (ab134113), expandable thumbnail
    Image from Cermak S et al., PLoS One. 2016;11(11):e0167428. Fig 3.; doi: 10.1371/journal.pone.0167428. Reproduced under the Creative Commons license http://creativecommons.org/licenses/by/4.0/

    Immunocytochemistry/ Immunofluorescence - Anti-Niemann Pick C1 antibody [EPR5209] (ab134113)

    Cathepsin B/L inhibition causes NPC disease-like cholesterol accumulation in SH-SY5Y cells.

    Confocal microscopy of SH-SY5Y control and PADK treated cells. Cholesterol (filipin staining, white) and NPC1 (ab134113; green).

  • Immunocytochemistry/ Immunofluorescence - Anti-Niemann Pick C1 antibody [EPR5209] (ab134113), expandable thumbnail
    Schultz et al Nat Commun. 2018; 9: 3671. Published online 2018 Sep 10. doi: 10.1038/s41467-018-06115-2

    Immunocytochemistry/ Immunofluorescence - Anti-Niemann Pick C1 antibody [EPR5209] (ab134113)

    I1061T NPC1 traffics to autophagosomes

    I1061T NPC1 fibroblasts were treated with vehicle or 100 nM bafilomycin A1 (Baf) for 24 h. Fixed cells were stained for LC3 (red), NPC1 (green), and DAPI (blue) then imaged by confocal microscopy. Co-localization is indicated by yellow color in the merged image. Scale bar = 25 μm.

    Niemann Pick C1 (NPC1) was detected using ab134113 at 1/200 dilution.

    From Figure 4b of Schultz et al.

    Shultz et al Nat Commun. 2018; 9: 3671.Published online 2018 Sep 10. doi: 10.1038/s41467-018-06115-2

    Reproduced under the Creative Commons Licence http://creativecommons.org/licenses/by/4.0/.

  • Western blot - Anti-Niemann Pick C1 antibody [EPR5209] (ab134113), expandable thumbnail

    Western blot - Anti-Niemann Pick C1 antibody [EPR5209] (ab134113)

    Lanes 1 - 4: Merged signal (red and green). Green - ab134113 observed at 180 kDa. Red - loading control, ab9484, observed at 37 kDa.

    ab134113 was shown to specifically react with Niemann Pick C1 in wild-type HAP1 cells as signal was lost in NPC1 (Niemann Pick C1) knockout cells. Wild-type and NPC1 (Niemann Pick C1) knockout samples were subjected to SDS-PAGE. Ab134113 and ab9484 (Mouse anti-GAPDH loading control) were incubated overnight at 4°C at 1/1000 dilution and 1/20000 dilution respectively. Blots were developed with Goat anti-Rabbit IgG H&L (IRDye® 800CW) preabsorbed ab216773 and Goat anti-Mouse IgG H&L (IRDye® 680RD) preabsorbed ab216776 secondary antibodies at 1/20000 dilution for 1 hour at room temperature before imaging.

    All lanes: Western blot - Anti-Niemann Pick C1 antibody [EPR5209] (AB134113) at 1/1000 dilution

    Lane 1: Wild-type HAP1 whole cell lysate at 20 µg

    Lane 2: NPC1 (Niemann Pick C1) knockout HAP1 whole cell lysate at 20 µg

    Lane 3: HEK293 whole cell lysate at 20 µg

    Lane 4: HepG2 whole cell lysate at 20 µg

    Predicted band size: 142 kDa

  • Immunocytochemistry/ Immunofluorescence - Anti-Niemann Pick C1 antibody [EPR5209] (ab134113), expandable thumbnail

    Immunocytochemistry/ Immunofluorescence - Anti-Niemann Pick C1 antibody [EPR5209] (ab134113)

    Immunofluorescent analysis of 4% paraformaldehyde-fixed, 0.1% Triton X-100 permeabilized Neuro-2a (mouse neuroblastoma cell line) cells labeling Niemann Pick C1 with ab134113 at 1/70 followed by Goat anti-rabbit IgG (Alexa Fluor® 488) (ab150077) secondary antibody at 1/500(green). Confocal image showing cytoplasmic staining on Neuro-2a cell line. The nuclear counterstain is DAPI (blue). Tubulin is detected with ab7291 (anti-Tubulin mouse mAb) at 1/1000 and ab150120(AlexaFluor®594 Goat anti-Mouse secondary) at 1/500 (red).

    The negative controls are as follows:
    -ve control 1 – ab134113 at 1/70 followed by ab150120 (AlexaFluor®594 Goat anti-Mouse secondary) at 1/500.
    -ve control 2. - ab7291 (anti-Tubulin mouse mAb) at 1/1000 followed by ab150077 (Alexa Fluor®488 Goat Anti-Rabbit IgG H&L) at 1/500.

  • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Niemann Pick C1 antibody [EPR5209] (ab134113), expandable thumbnail

    Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Niemann Pick C1 antibody [EPR5209] (ab134113)

    Immunohistochemical staining of paraffin embedded human liver with purified ab134113 at a working dilution of 1 in 50. The secondary antibody used is a HRP polymer for rabbit IgG. The sample is counter-stained with hematoxylin. Antigen retrieval was perfomed using Tris-EDTA buffer, pH 9.0. PBS was used instead of the primary antibody as the negative control, and is shown in the inset.

  • Flow Cytometry (Intracellular) - Anti-Niemann Pick C1 antibody [EPR5209] (ab134113), expandable thumbnail

    Flow Cytometry (Intracellular) - Anti-Niemann Pick C1 antibody [EPR5209] (ab134113)

    ab134113 staining Niemann Pick C1 in Neuro-2a (mouse neuroblastoma cell line) by intracellular flow cytometry. Cells were fixed with 4% paraformaldehyde, permabilised with 90% methanol and the sample was incubated with the primary antibody at a dilution of 1/200. A goat anti rabbit IgG (Alexa Fluor® 488) at a dilution of 1/2000 was used as the secondary antibody.

    Isoytype control: Rabbit monoclonal IgG (Black)

    Unlabeled control: Cell without incubation with primary antibody and secondary antibody (Blue)

  • Western blot - Anti-Niemann Pick C1 antibody [EPR5209] (ab134113), expandable thumbnail

    Western blot - Anti-Niemann Pick C1 antibody [EPR5209] (ab134113)

    Blocking buffer: 5% NFDM/TBST

    Dilution buffer: 5% NFDM/TBST

    All lanes: Western blot - Anti-Niemann Pick C1 antibody [EPR5209] (AB134113) at 1/10000 dilution

    Lane 1: HepG2 (human liver hepatocellular carcinoma cell line) cell lysate at 20 µg

    Lane 2: THP-1 (human monocytic leukemia cell line) cell lysate at 20 µg

    Lane 3: HEK-293 (human epithelial cell line from embryonic kidney) cell lysate at 20 µg

    Lane 4: PC-3 (human prostate adenocarcinoma cell line) cell lysate at 20 µg

    Secondary

    All lanes: HRP goat anti-rabbit (H+L) at 1/1000 dilution

    Predicted band size: 142 kDa

    Observed band size: 180 kDa

  • Western blot - Anti-Niemann Pick C1 antibody [EPR5209] (ab134113), expandable thumbnail

    Western blot - Anti-Niemann Pick C1 antibody [EPR5209] (ab134113)

    Blocking buffer: 5% NFDM/TBST

    Dilution buffer: 5% NFDM/TBST

    All lanes: Western blot - Anti-Niemann Pick C1 antibody [EPR5209] (AB134113) at 1/10000 dilution

    Lane 1: 3T3-L1 cell lysate at 20 µg

    Lane 2: L6 (rat skeletal muscle cell line) cell lysate at 20 µg

    Lane 3: Rat tissue lysate at 20 µg

    Secondary

    All lanes: HRP goat anti-rabbit (H+L) at 1/1000 dilution

    Predicted band size: 142 kDa

    Observed band size: 180 kDa

  • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Niemann Pick C1 antibody [EPR5209] (ab134113), expandable thumbnail

    Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Niemann Pick C1 antibody [EPR5209] (ab134113)

    Immunohistochemical staining of paraffin embedded mouse liver with purified ab134113 at a working dilution of 1 in 50. The secondary antibody used is a HRP polymer for rabbit IgG. The sample is counter-stained with hematoxylin. Antigen retrieval was perfomed using Tris-EDTA buffer, pH 9.0. PBS was used instead of the primary antibody as the negative control, and is shown in the inset.

  • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Niemann Pick C1 antibody [EPR5209] (ab134113), expandable thumbnail

    Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Niemann Pick C1 antibody [EPR5209] (ab134113)

    Immunohistochemical staining of paraffin embedded rat cerebellum with purified ab134113 at a working dilution of 1 in 50. The secondary antibody used is a HRP polymer for rabbit IgG. The sample is counter-stained with hematoxylin. Antigen retrieval was perfomed using Tris-EDTA buffer, pH 9.0. PBS was used instead of the primary antibody as the negative control, and is shown in the inset.

  • Western blot - Anti-Niemann Pick C1 antibody [EPR5209] (ab134113), expandable thumbnail

    Western blot - Anti-Niemann Pick C1 antibody [EPR5209] (ab134113)

    All lanes: Western blot - Anti-Niemann Pick C1 antibody [EPR5209] (AB134113) at 1/1000 dilution

    Lane 1: 3T3 L1 cell lysate at 10 µg

    Lane 2: L6 (rat skeletal muscle cell line) cell lysate at 10 µg

    Lane 3: HepG2 (human liver hepatocellular carcinoma cell line) cell lysate at 10 µg

    Lane 4: THP-1 (human monocytic leukemia cell line) cell lysate at 10 µg

    Lane 5: HEK-293T (human epithelial cell line from embryonic kidney transformed with large T antigen) cell lysate at 10 µg

    Lane 6: PC-3 (human prostate adenocarcinoma cell line) cell lysate at 10 µg

    Lane 7: Rat liver lysate at 10 µg

    Lane 8: Rat brain lysate at 10 µg

    Secondary

    All lanes: Goat anti-rabbit HRP conjugated antibody at 1/2000 dilution

    Predicted band size: 142 kDa

    Observed band size: 180 kDa

  • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Niemann Pick C1 antibody [EPR5209] (ab134113), expandable thumbnail

    Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Niemann Pick C1 antibody [EPR5209] (ab134113)

    Immunohistochemical analysis of paraffin embedded human kidney tissue labelling Niemann Pick C1 with unpurified ab134113 at 1/50.

    Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.

  • OI-RD Scanning - Anti-Niemann Pick C1 antibody [EPR5209] (ab134113), expandable thumbnail

    OI-RD Scanning - Anti-Niemann Pick C1 antibody [EPR5209] (ab134113)

    We have systematically measured KD (the equilibrium dissociation constant between the antibody and its antigen), of more than 840 recombinant antibodies to assess not only their individual KD values but also to see the average affinity of antibody.
    Based on the comparison with published literature values for mouse monoclonal antibodies, Recombinant antibodies appear to be on average 1-2 order of magnitude higher affinity.

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