Overview

  • Product name

  • Description

    Rabbit polyclonal to PEX5/PER3
  • Host species

    Rabbit
  • Tested applications

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

    Reacts with: Human
    Predicted to work with: Mouse, Rat, Guinea pig, Cow, Chinese hamster
  • Immunogen

    Recombinant fragment corresponding to Human PEX5/PER3 aa 237-330.
    Sequence:

    RAQAEQWAAEFIQQQGTSDAWVDQFTRPVNTSALDMEFERAKSAIESDVD FWDKLQAELEEMAKRDAEAHPWLSDYDDLTSATYDKGYQFEEEN


    Database link: P50542

  • Positive control

    • Human testis tissue; U-2 OS cells; RT-4, U-251 MG and Human liver lysates.
  • General notes

     This product was previously labelled as PEX5

     

Properties

  • Form

    Liquid
  • Storage instructions

    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
  • Storage buffer

    pH: 7.2
    Preservative: 0.02% Sodium azide
    Constituents: 59% PBS, 40% Glycerol
  • Concentration information loading...
  • Purity

    Immunogen affinity purified
  • Clonality

    Polyclonal
  • Isotype

    IgG
  • Research areas

Applications

Our Abpromise guarantee covers the use of ab204444 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.
WB 1/100 - 1/250. Predicted molecular weight: 71 kDa.
ICC/IF Use a concentration of 1 - 4 µg/ml.

Fixation/Permeabilization: PFA/Triton X-100.

Target

  • Function

    Binds to the C-terminal PTS1-type tripeptide peroxisomal targeting signal (SKL-type) and plays an essential role in peroxisomal protein import.
  • Tissue specificity

    Detected in heart, brain, placenta, lung, liver, skeletal muscle, kidney and pancreas.
  • Involvement in disease

    Defects in PEX5 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. Inheritance is autosomal recessive.
    Defects in PEX5 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.
    Defects in PEX5 may be a cause of infantile Refsum disease (IRD) [MIM:266510]. IRD is a mild peroxisome biogenesis disorder (PBD). Clinical features include early onset, mental retardation, minor facial dysmorphism, retinopathy, sensorineural hearing deficit, hepatomegaly, osteoporosis, failure to thrive, and hypocholesterolemia. The biochemical abnormalities include accumulation of phytanic acid, very long chain fatty acids (VLCFA), di- and trihydroxycholestanoic acid and pipecolic acid.
  • Sequence similarities

    Belongs to the peroxisomal targeting signal receptor family.
    Contains 7 TPR repeats.
  • Cellular localization

    Cytoplasm. Peroxisome membrane. Its distribution appears to be dynamic. It is probably a cycling receptor found mainly in the cytoplasm and as well associated to the peroxisomal membrane through a docking factor.
  • Information by UniProt
  • Database links

  • Alternative names

    • FLJ50634 antibody
    • FLJ50721 antibody
    • FLJ51948 antibody
    • PBD2A antibody
    • PBD2B antibody
    • Peroxin 5 antibody
    • Peroxin-5 antibody
    • Peroxisomal biogenesis factor 5 antibody
    • Peroxisomal C terminal targeting signal import receptor antibody
    • Peroxisomal C-terminal targeting signal import receptor antibody
    • Peroxisomal targeting signal 1 (SKL type) receptor antibody
    • Peroxisomal targeting signal 1 receptor antibody
    • Peroxisomal targeting signal import receptor antibody
    • Peroxisomal targeting signal receptor 1 antibody
    • Peroxisome receptor 1 antibody
    • pex5 antibody
    • PEX5_HUMAN antibody
    • PTS1 BP antibody
    • PTS1 receptor antibody
    • PTS1-BP antibody
    • PTS1R antibody
    • PXR1 antibody
    see all

Images

  • All lanes : Anti-PEX5/PER3 antibody (ab204444) at 1/100 dilution

    Lane 1 : RT-4 cell lysate
    Lane 2 : U-251 MG cell lysate
    Lane 3 : Human plasma lysate
    Lane 4 : Human liver lysate

    Predicted band size: 71 kDa

  • Immunofluorescent analysis of PFA-fixed, Triton X-100 permeabilized U-2 OS cells labeling PEX5/PER3 with ab204444 at 4µg/ml (green).

  • Immunohistochemical analysis of paraffin-embedded Human testis tissue labeling PEX5/PER3 with ab204444 at 1/50 dilution.

References

ab204444 has not yet been referenced specifically in any publications.

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