Overview

  • Product name

  • Description

    Rabbit polyclonal to PEX13
  • Host species

    Rabbit
  • Tested applications

    Suitable for: IHC-P, ICC/IFmore details
  • Species reactivity

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

    Recombinant fragment corresponding to Human PEX13 aa 324-398.
    Sequence:

    GLIPANYVKILGKRKGRKTVESSKVSKQQQSFTNPTLTKGATVADSLDEQ EAAFESVFVETNKVPVAPDSIGKDG


    Database link: Q92968

  • Positive control

    • Human rectum tissue; U-2 OS cells.

Properties

Applications

Our Abpromise guarantee covers the use of ab204841 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
IHC-P 1/50 - 1/200. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.
ICC/IF Use a concentration of 1 - 4 µg/ml.

Fixation/Permeabilization: PFA/Triton X-100

Target

  • Function

    Component of the peroxisomal translocation machinery with PEX14 and PEX17. Functions as a docking factor for the predominantly cytoplasmic PTS1 receptor (PAS10/PEX5). Involved in the import of PTS1 and PTS2 proteins.
  • Involvement in disease

    Defects in PEX13 are the cause of peroxisome biogenesis disorder complementation group 13 (PBD-CG13) [MIM:601789]; also known as PBD-CGH. PBD-CG13 is a peroxisomal disorder arising from a failure of protein import into the peroxisomal membrane or matrix. The peroxisome biogenesis disorders (PBD group) are genetically heterogeneous with at least 14 distinct genetic groups as concluded from complementation studies. Include disorders are: Zellweger syndrome (ZWS), neonatal adrenoleukodystrophy (NALD), infantile Refsum disease (IRD), and classical rhizomelic chondrodysplasia punctata (RCDP). ZWS, NALD and IRD are distinct from RCDP and constitute a clinical continuum of overlapping phenotypes known as the Zellweger spectrum (PBD-ZSS).
    Defects in PEX13 are a cause of adrenoleukodystrophy neonatal (NALD) [MIM:202370]. NALD is a peroxisome biogenesis disorder (PBD) characterized by the accumulation of very long-chain fatty acids, adrenal insufficiency and mental retardation.
  • Sequence similarities

    Belongs to the peroxin-13 family.
    Contains 1 SH3 domain.
  • Cellular localization

    Peroxisome membrane.
  • Information by UniProt
  • Database links

  • Alternative names

    • 2610008O20Rik antibody
    • NALD antibody
    • OTTMUSP00000005454 antibody
    • Peroxin 13 antibody
    • Peroxin-13 antibody
    • Peroxin13 antibody
    • Peroxisomal membrane protein PEX 13 antibody
    • Peroxisomal membrane protein PEX13 antibody
    • Peroxisome biogenesis factor 13 antibody
    • PEX 13 antibody
    • pex13 antibody
    • PEX13_HUMAN antibody
    • RP23-188K3.1 antibody
    • ZWS antibody
    see all

Images

  • Immunofluorescent analysis of U-2 OS cells labeling PEX13 with ab204841 at 4 µg/ml.

  • Immunohistochemical analysis of formalin-fixed paraffin-embedded Human rectum tissue labeling PEX13 with ab204841 at 1/50.

References

ab204841 has not yet been referenced specifically in any publications.

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