Description

  • Product name

    Recombinant Human PEX13 protein
  • Expression system

    Wheat germ
  • Accession

  • Protein length

    Full length protein
  • Animal free

    No
  • Nature

    Recombinant
    • Species

      Human
    • Sequence

      MASQPPPPPKPWETRRIPGAGPGPGPGPTFQSADLGPTLMTRPGQPALTR VPPPILPRPSQQTGSSSVNTFRPAYSSFSSGYGAYGNSFYGGYSPYSYGY NGLGYNRLRVDDLPPSRFVQQAEESSRGAFQSIESIVHAFASVSMMMDAT FSAVYNSFRAVLDVANHFSRLKIHFTKVFSAFALVRTIRYLYRRLQRMLG LRRGSENEDLWAESEGTVACLGAEDRAATSAKSWPIFLFFAVILGGPYLI WKLLSTHSDEVTDSINWASGEDDHVVARAEYDFAAVSEEEISFRAGDMLN LALKEQQPKVRGWLLASLDGQTTGLIPANYVKILGKRKGRKTVESSKVSK QQQSFTNPTLTKGATVADSLDEQEAAFESVFVETNKVPVAPDSIGKDGEK QDL
    • Predicted molecular weight

      71 kDa including tags
    • Amino acids

      1 to 403

Specifications

Our Abpromise guarantee covers the use of ab131942 in the following tested applications.

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

  • Applications

    Western blot

    SDS-PAGE

    ELISA

  • Form

    Liquid
  • Additional notes

    Protein concentration is above or equal to 0.05 µg/µl.
    Best use within three months from the date of receipt of this protein.
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.31% Glutathione, 0.79% Tris HCl

General Info

  • Alternative names

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

Images

  • 12.5% SDS-PAGE showing ab131942 at approximately 70.5 kDa.
    Stained with Coomassie Blue.

References

ab131942 has not yet been referenced specifically in any publications.

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