Overview

  • Product name
    Anti-Niemann Pick C1 antibody
    See all Niemann Pick C1 primary antibodies
  • Description
    Goat polyclonal to Niemann Pick C1
  • 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

  • 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

  • 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.

Customer reviews and Q&As

There are currently no Customer reviews or Questions for ab177233.
Please use the links above to contact us or submit feedback about this product.

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

Sign up