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Anti-Niemann Pick C1 antibody (ab177233)

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Western blot - Anti-Niemann Pick C1 antibody (ab177233)

    Key features and details

    • Goat polyclonal to Niemann Pick C1
    • Suitable for: WB
    • Reacts with: Human
    • Isotype: IgG

    Get better batch-to-batch reproducibility with a recombinant antibody

    Product image
    Anti-Niemann Pick C1 antibody [EPR5209] (ab134113)
    • Research with confidence – consistent and reproducible results with every batch
    • Long-term and scalable supply – powered by recombinant technology for fast production
    • Success from the first experiment – confirmed specificity through extensive validation
    • Ethical standards compliant – production is animal-free

    Overview

    • Product name

      Anti-Niemann Pick C1 antibody
      See all Niemann Pick C1 primary antibodies
    • Description

      Goat polyclonal to Niemann Pick C1
    • Host species

      Goat
    • Tested applications

      Suitable for: WBmore details
    • Species reactivity

      Reacts with: Human
      Predicted to work with: Pig
    • Immunogen

      Synthetic peptide corresponding to Human Niemann Pick C1 aa 1040-1053 (internal sequence) (Cysteine residue).
      Sequence:

      TYHTVLQTSADFID


      Database link: NP_000262.2
      Run BLAST with BLAST the sequence with ExPASy Run BLAST with BLAST the sequence with NCBI
    • Positive control

      • HEK293 cell lysate.
    • General notes

      The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.

      If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, along with publications, customer reviews and Q&As

    Properties

    • Form

      Liquid
    • Storage instructions

      Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
    • Storage buffer

      pH: 7.30
      Preservative: 0.02% Sodium azide
      Constituents: 99% Tris buffered saline, 0.5% BSA
    • Concentration information loading...
    • Purity

      Immunogen affinity purified
    • Purification notes

      ab177233 was purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.
    • Clonality

      Polyclonal
    • Isotype

      IgG
    • Research areas

      • Neuroscience
      • Neurotransmitter
      • Transporters
      • Other
      • Cardiovascular
      • Lipids / Lipoproteins
      • Lipid Metabolism
      • Cholesterol Metabolism
      • Signal Transduction
      • Protein Trafficking
      • Vesicle Transport
      • Coat Proteins
      • Neuroscience
      • Neurology process
      • Neurodegenerative disease
      • Other
      • Cardiovascular
      • Atherosclerosis
      • Lipoprotein metabolism
      • Cardiovascular
      • Lipids / Lipoproteins
      • Fatty Acids
      • Metabolism
      • Metabolism
      • Pathways and Processes
      • Metabolic signaling pathways
      • Lipid and lipoprotein metabolism
      • Cholesterol Metabolism
      • Metabolism
      • Pathways and Processes
      • Metabolic signaling pathways
      • Lipid and lipoprotein metabolism
      • Lipoprotein metabolism
      • Metabolism
      • Pathways and Processes
      • Metabolic signaling pathways
      • Lipid and lipoprotein metabolism
      • Fatty acids
      • Metabolism
      • Types of disease
      • Heart disease
      • Metabolism
      • Pathways and Processes
      • Redox metabolism
      • Fatty acid oxidation

    Associated products

    • Compatible Secondaries

      • Donkey Anti-Goat IgG H&L (Alexa Fluor® 488) (ab150129)
      • Donkey Anti-Goat IgG H&L (HRP) (ab205723)
    • Isotype control

      • Goat IgG, polyclonal - Isotype Control (ab37373)
    • Recombinant Protein

      • Recombinant Human Niemann Pick C1 protein (ab114306)
    • Related Products

      • HEK-293 whole cell lysate (ab7902)

    Applications

    The Abpromise guarantee

    Our Abpromise guarantee covers the use of ab177233 in the following tested applications.

    The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

    Application Abreviews Notes
    WB
    Use a concentration of 0.5 - 1.5 µg/ml. Detects a band of approximately 170 kDa (predicted molecular weight: 142 kDa).

    1 hour primary incubation is recommended for this product.

    Notes
    WB
    Use a concentration of 0.5 - 1.5 µg/ml. Detects a band of approximately 170 kDa (predicted molecular weight: 142 kDa).

    1 hour primary incubation is recommended for this product.

    Target

    • Function

      Involved in the intracellular trafficking of 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.
    • Involvement in disease

      Defects in NPC1 are the cause of Niemann-Pick disease type C1 (NPDC1) [MIM:257220]. A lysosomal storage disorder that affects the viscera and the central nervous system. It is due to defective intracellular processing and transport of low-density lipoprotein derived cholesterol. It causes accumulation of cholesterol in lysosomes, with delayed induction of cholesterol homeostatic reactions. Niemann-Pick disease type C1 has a highly variable clinical phenotype. Clinical features include variable hepatosplenomegaly and severe progressive neurological dysfunction such as ataxia, dystonia and dementia. The age of onset can vary from infancy to late adulthood. An allelic variant of Niemann-Pick disease type C1 is found in people with Nova Scotia ancestry. Patients with the Nova Scotian clinical variant are less severely affected.
    • Sequence similarities

      Belongs to the patched family.
      Contains 1 SSD (sterol-sensing) domain.
    • Domain

      A cysteine-rich N-terminal domain and a C-terminal domain containing a di-leucine motif necessary for lysosomal targeting are critical for mobilization of cholesterol from lysosomes.
    • Post-translational
      modifications

      Glycosylated.
    • Cellular localization

      Late endosome membrane. Lysosome membrane.
    • Target information above from: UniProt accession O15118 The UniProt Consortium
      The Universal Protein Resource (UniProt) in 2010
      Nucleic Acids Res. 38:D142-D148 (2010) .

      Information by UniProt
    • Database links

      • Entrez Gene: 4864 Human
      • Entrez Gene: 397591 Pig
      • Omim: 607623 Human
      • SwissProt: O15118 Human
      • SwissProt: P56941 Pig
      • Unigene: 464779 Human
      • Unigene: 715623 Human
      • Alternative names

        • Niemann Pick C1 protein precursor antibody
        • Niemann Pick disease, type C1 antibody
        • Niemann-Pick C1 protein antibody
        • NPC antibody
        • NPC1 antibody
        • NPC1_HUMAN antibody
        see all

      Images

      • Western blot - Anti-Niemann Pick C1 antibody (ab177233)
        Western blot - Anti-Niemann Pick C1 antibody (ab177233)
        Anti-Niemann Pick C1 antibody (ab177233) at 0.5 µg/ml + HEK293 cell lysate (in RIPA buffer) at 35 µg

        Developed using the ECL technique.

        Predicted band size: 142 kDa
        Observed band size: 170 kDa why is the actual band size different from the predicted?



        Primary incubation was 1 hour.

      Protocols

      • Western blot protocols

      Click here to view the general protocols

      Datasheets and documents

      • SDS download

      • Datasheet download

        Download

      References (0)

      Publishing research using ab177233? Please let us know so that we can cite the reference in this datasheet.

      ab177233 has not yet been referenced specifically in any publications.

      Customer reviews and Q&As

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