Overview

  • Product name
  • Description
    Rabbit polyclonal to PEX7
  • Host species
    Rabbit
  • Tested applications
    Suitable for: WB, IHC-Pmore details
  • Species reactivity
    Reacts with: Mouse, Rat, Human
  • Immunogen

    Synthetic peptide derived from internal sequence of Human PEX7.

  • Positive control
    • NIH-3T3 cell extract; Human breast tissue.

Properties

  • Form
    Liquid
  • Storage instructions
    Shipped at 4°C. Store at -20ºC.
  • Storage buffer
    pH: 7.40
    Preservative: 0.02% Sodium azide
    Constituents: 49% PBS, 50% Glycerol, 0.88% Sodium chloride
    Note: PBS without Mg2+ and Ca2+.
  • Concentration information loading...
  • Purity
    Immunogen affinity purified
  • Clonality
    Polyclonal
  • Isotype
    IgG
  • Research areas

Applications

Our Abpromise guarantee covers the use of ab167036 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 1/500 - 1/1000. Predicted molecular weight: 36 kDa.
IHC-P Use a concentration of 5 µg/ml.

Target

  • Function
    Binds to the N-terminal PTS2-type peroxisomal targeting signal and plays an essential role in peroxisomal protein import.
  • Tissue specificity
    Ubiquitous. Highest expression in pancreas, skeletal muscle and heart.
  • Involvement in disease
    Defects in PEX7 are the cause of peroxisome biogenesis disorder complementation group 11 (PBD-CG11) [MIM:614879]. 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 13 distinct genetic groups as concluded from complementation studies.
    Defects in PEX7 are the cause of rhizomelic chondrodysplasia punctata type 1 (RCDP1) [MIM:215100]. RCDP1 is characterized by rhizomelic shortening of femur and humerus, vertebral disorders, cataract, cutaneous lesions and severe mental retardation.
    Defects in PEX7 are the cause of peroxisome biogenesis disorder 9B (PBD9B) [MIM:614879]. A peroxisome biogenesis disorder with unusually mild clinical and biochemical manifestations. Affected individuals manifest a variable phenotype similar to, and in some cases indistinguishable from, classic Refsum disease. Variable features include ocular abnormalities, sensorimotor neuropathy, ichthyosis, deafness, chondrodysplasia punctata without rhizomelia or growth failure.
  • Sequence similarities
    Belongs to the WD repeat peroxin-7 family.
    Contains 6 WD repeats.
  • Cellular localization
    Peroxisome. Cytoplasm.
  • Information by UniProt
  • Database links
  • Alternative names
    • PBD9B antibody
    • PCDP1 antibody
    • Peroxin 7 antibody
    • Peroxin-7 antibody
    • Peroxisomal PTS2 receptor antibody
    • Peroxisomal targeting signal 2 receptor antibody
    • Peroxisome biogenesis factor 7 antibody
    • Peroxisome targeting signal 2 receptor antibody
    • PEX7 antibody
    • PEX7 protein antibody
    • PEX7_HUMAN antibody
    • PTS2 receptor antibody
    • PTS2R antibody
    • RCDP1 antibody
    • RD antibody
    see all

Images

  • Immunohistochemical analysis of formalin-fixed, paraffin-embedded Human Breast tissue labeling PEX7 with ab167036 at 5 µg/ml.
  • All lanes : Anti-PEX7 antibody (ab167036) at 1/500 dilution

    Lane 1 : NIH-3T3 cell extract
    Lane 2 : NIH-3T3 cell extract with immunizing peptide

    Predicted band size: 36 kDa

References

ab167036 has not yet been referenced specifically in any publications.

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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