Overview

  • Product nameAnti-PEX19 antibody
    See all PEX19 primary antibodies
  • Description
    Rabbit polyclonal to PEX19
  • Tested applicationsSuitable for: WBmore details
  • Species reactivity
    Reacts with: Human
  • Immunogen

    Synthetic peptide conjugated to KLH, corresponding to a region within N terminal amino acids 32-61 of Human PEX19 (NP_002848.1, NP_001180573.1).

  • Positive control
    • HeLa cell lysate.

Properties

Applications

Our Abpromise guarantee covers the use of ab123315 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 1/100 - 1/500. Predicted molecular weight: 33 kDa.

Target

  • FunctionNecessary for early peroxisomal biogenesis. Acts both as a cytosolic chaperone and as an import receptor for peroxisomal membrane proteins (PMPs). Binds and stabilizes newly synthesized PMPs in the cytoplasm by interacting with their hydrophobic membrane-spanning domains, and targets them to the peroxisome membrane by binding to the integral membrane protein PEX3. Excludes CDKN2A from the nucleus and prevents its interaction with MDM2, which results in active degradation of TP53.
  • Tissue specificityUbiquitously expressed. Isoform 1 is strongly predominant in all tissues except in utero where isoform 2 is the main form.
  • Involvement in diseaseDefects in PEX19 are the cause of peroxisome biogenesis disorder complementation group 14 (PBD-CG14) [MIM:600279]; also known as PBD-CGJ. PBD refers to a group of peroxisomal disorders arising from a failure of protein import into the peroxisomal membrane or matrix. The PBD group is comprised of four disorders: 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. The PBD group is genetically heterogeneous with at least 14 distinct genetic groups as concluded from complementation studies.
    Defects in PEX19 are a cause of Zellweger syndrome (ZWS) [MIM:214100]. ZWS is a fatal peroxisome biogenesis disorder characterized by dysmorphic facial features, hepatomegaly, ocular abnormalities, renal cysts, hearing impairment, profound psychomotor retardation, severe hypotonia and neonatal seizures. Death occurs within the first year of life.
  • Sequence similaritiesBelongs to the peroxin-19 family.
  • Cellular localizationCytoplasm. Peroxisome membrane. Mainly cytoplasmic. Some fraction membrane-associated to the outer surface of peroxisomes.
  • Information by UniProt
  • Database links
  • Alternative names
    • 33 kDa housekeeping protein antibody
    • D1S2223E antibody
    • HK33 antibody
    • Housekeeping gene 33kD antibody
    • OK/SW-cl.22 antibody
    • PBD12A antibody
    • Peroxin 19 antibody
    • Peroxin-19 antibody
    • Peroxisomal biogenesis factor 19 antibody
    • Peroxisomal farnesylated protein antibody
    • PEX19 antibody
    • PEX19_HUMAN antibody
    • PMP1 antibody
    • PMPI antibody
    • PXF antibody
    • PXMP1 antibody
    see all

Anti-PEX19 antibody images

  • Anti-PEX19 antibody (ab123315) at 1/100 dilution + HeLa cell lysate at 35 µg
    Developed using the ECL technique

    Predicted band size : 33 kDa

References for Anti-PEX19 antibody (ab123315)

ab123315 has not yet been referenced specifically in any publications.

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