Overview

  • Product name

    Anti-ARH antibody [EPR13116] - C-terminal
    See all ARH primary antibodies
  • Description

    Rabbit monoclonal [EPR13116] to ARH - C-terminal
  • Host species

    Rabbit
  • Tested applications

    Suitable for: WBmore details
    Unsuitable for: Flow Cyt,ICC/IF,IHC-P or IP
  • Species reactivity

    Reacts with: Human
  • Immunogen

    Synthetic peptide (the amino acid sequence is considered to be commercially sensitive) within Human ARH aa 250 to the C-terminus (Cysteine residue). The exact sequence is proprietary.
    Database link: Q5SW96

  • Positive control

    • Human fetal liver, HeLa, K562 and 293T cell lysates.
  • General notes

    Mouse, Rat: We have preliminary internal testing data to indicate this antibody may not react with these species. Please contact us for more information.

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

    We are constantly working hard to ensure we provide our customers with best in class antibodies. As a result of this work we are pleased to now offer this antibody in purified format. We are in the process of updating our datasheets. The purified format is designated 'PUR' on our product labels. If you have any questions regarding this update, please contact our Scientific Support team.

    This product is a recombinant rabbit monoclonal antibody.

Properties

Applications

Our Abpromise guarantee covers the use of ab179828 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 1/10000 - 1/50000. Predicted molecular weight: 34 kDa.
  • Application notes
    Is unsuitable for Flow Cyt,ICC/IF,IHC-P or IP.
  • Target

    • Function

      Adapter protein (clathrin-associated sorting protein (CLASP)) required for efficient endocytosis of the LDL receptor (LDLR) in polarized cells such as hepatocytes and lymphocytes, but not in non-polarized cells (fibroblasts). May be required for LDL binding and internalization but not for receptor clustering in coated pits. May facilitate the endocytocis of LDLR and LDLR-LDL complexes from coated pits by stabilizing the interaction between the receptor and the structural components of the pits. May also be involved in the internalization of other LDLR family members. Binds to phosphoinositides, which regulate clathrin bud assembly at the cell surface.
    • Tissue specificity

      Expressed at high levels in the kidney, liver, and placenta, with lower levels detectable in brain, heart, muscle, colon, spleen, intestine, lung, and leukocytes.
    • Involvement in disease

      Defects in LDLRAP1 are the cause of autosomal recessive hypercholesterolemia (ARH) [MIM:603813]. ARH is a disorder caused by defective internalization of LDL receptors (LDLR) in the liver. ARH has the clinical features of familial hypercholesterolemia (FH) [MIM:143890] homozygotes, including severely elevated plasma LDL cholesterol, tuberous and tendon xanthomata, and premature atherosclerosis. LDL receptor (LDLR) activity measured in skin fibroblasts is normal, as the LDL binding ability.
    • Sequence similarities

      Contains 1 PID domain.
    • Domain

      The [DE]-X(1,2)-F-X-X-[FL]-X-X-X-R motif mediates interaction the AP-2 complex subunit AP2B1.
    • Post-translational
      modifications

      Phosphorylated upon DNA damage, probably by ATM or ATR.
    • Cellular localization

      Cytoplasm.
    • Information by UniProt
    • Database links

    • Alternative names

      • ARH antibody
      • ARH GENE antibody
      • ARH_HUMAN antibody
      • ARH1 antibody
      • ARH2 antibody
      • Autosomal recessive hypercholesterolemia protein antibody
      • DKFZp586D0624 antibody
      • FHCB1 antibody
      • FHCB2 antibody
      • LDL receptor adaptor protein antibody
      • Ldlrap1 antibody
      • Low density lipoprotein receptor adapter protein 1 antibody
      • MGC34705 antibody
      • OTTHUMP00000008526 antibody
      see all

    Images

    • All lanes : Anti-ARH antibody [EPR13116] - C-terminal (ab179828) at 1/10000 dilution

      Lane 1 : Human fetal liver lysate
      Lane 2 : HeLa cell lysate
      Lane 3 : K562 cell lysate
      Lane 4 : 293T cell lysate

      Lysates/proteins at 10 µg per lane.

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

      Predicted band size: 34 kDa

    References

    This product has been referenced in:

    • Rodríguez-Jiménez C  et al. A new variant (c.1A>G) in LDLRAP1 causing autosomal recessive hypercholesterolemia: Characterization of the defect and response to PCSK9 inhibition. Atherosclerosis N/A:N/A (2019). Read more (PubMed: 30777337) »
    See 1 Publication for this product

    Customer reviews and Q&As

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