Overview

  • Product nameRecombinant Human PEX19 protein
  • Protein lengthFull length protein

Description

  • NatureRecombinant
  • SourceEscherichia coli
  • Amino Acid Sequence
    • AccessionP40855
    • SpeciesHuman
    • SequenceMGSSHHHHHHSSGLVPRGSHMAAAEEGCSVGAEADRELEELLESALDDFD KAKPSPAPPSTTTAPDASGPQKRSPGDTAKDALFASQEKFFQELFDSELA SQATAEFEKAMKELAEEEPHLVEQFQKLSEAAGRVGSDMTSQQEFTSCLK ETLSGLAKNATDLQNSSMSEEELTKAMEGLGMDEGDGEGNILPIMQSIMQ NLLSKDVLYPSLKEITEKYPEWLQSHRESLPPEQFEKYQEQHSVMCKICE QFEAETPTDSETTQKARFEMVLDLMQQLQDLGHPPKELAGEMPPGLNFDL DALNLSGPPGASGEQC
    • Molecular weight35 kDa including tags
    • Amino acids1 to 296
    • TagsHis tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab111623 in the following tested applications.

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

  • Applications

    SDS-PAGE

    Mass Spectrometry

  • Mass spectrometry
    MALDI-TOF
  • Purity> 90 % SDS-PAGE.
    ab111623 is purified using conventional chromatography techniques.
  • FormLiquid
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.

    pH: 8.00
    Constituents: 10% Glycerol, 0.32% Tris HCl

General Info

  • Alternative names
    • 33 kDa housekeeping protein
    • D1S2223E
    • HK33
    • Housekeeping gene 33kD
    • OK/SW-cl.22
    • PBD12A
    • Peroxin 19
    • Peroxin-19
    • Peroxisomal biogenesis factor 19
    • Peroxisomal farnesylated protein
    • PEX19
    • PEX19_HUMAN
    • PMP1
    • PMPI
    • PXF
    • PXMP1
    see all
  • 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

Recombinant Human PEX19 protein images

  • 15% SDS-PAGE showing ab111623 at approximately 34.6kDa (3µg).
    (Molecular weight on SDS-PAGE will appear higher)

References for Recombinant Human PEX19 protein (ab111623)

ab111623 has not yet been referenced specifically in any publications.

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