Key features and details
- Mouse polyclonal to PEX19
- Suitable for: WB
- Reacts with: Human
- Isotype: IgG
Product nameAnti-PEX19 antibody
See all PEX19 primary antibodies
DescriptionMouse polyclonal to PEX19
Tested applicationsSuitable for: WBmore details
Species reactivityReacts with: Human
Full length human PEX19 protein (NP_002848.1)
- Jurkat cell lysate 293T transfected cell lysate
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Storage instructionsShipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
Storage bufferpH: 7.4
Constituent: 2.68% PBS
Concentration information loading...
PurityProtein A purified
Our Abpromise guarantee covers the use of ab88365 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||Use a concentration of 1 µg/ml. Predicted molecular weight: 33 kDa.|
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
- 33 kDa housekeeping protein antibody
- D1S2223E antibody
- HK33 antibody
Anti-PEX19 antibody (ab88365) at 1 µg/ml + Jurkat cell lysate at 50 µg
Goat anti-mouse IgG at 1/5000 dilution
Predicted band size: 33 kDa
All lanes : Anti-PEX19 antibody (ab88365) at 1 µg/ml
Lane 1 : PEX19 transfected 293T cell lysate
Lane 2 : Untransfected 293T cell lysate
Lysates/proteins at 50 µg per lane.
All lanes : Goat anti mouse IgG at 1/5000 dilution
Predicted band size: 33 kDa
Observed band size: 33 kDa
ab88365 has not yet been referenced specifically in any publications.