Anti-Niemann Pick C1 antibody (ab177233)

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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.

Properties

Applications

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).

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.
  • Information by UniProt

  • Database links

    • 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.

    References

    ab177233 has not yet been referenced specifically in any publications.

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

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