Overview

  • Product name
    Anti-PEX2 antibody - C-terminal
    See all PEX2 primary antibodies
  • Description
    Mouse polyclonal to PEX2 - C-terminal
  • Host species
    Mouse
  • Tested applications
    Suitable for: WB, ELISAmore details
  • Species reactivity
    Reacts with: Mouse, Human
    Predicted to work with: Rat, Chinese hamster
  • Immunogen

    Recombinant fragment (GST-tag) corresponding to Human PEX2 aa 217-305 (C terminal).
    Sequence:

    KAKLSSWCIPLTGAPNSDNTLATSGKECALCGEWPTMPHTIGCEHIFCYF CAKSSFLFDVYFTCPKCGTEVHSLQPLKSGIEMSEVNAL


    Database link: NP_000309

  • Positive control
    • PEX2 human recombinant protein fragment; HeLa cell lysate; NIH3T3 cell lysate.

Properties

Applications

Our Abpromise guarantee covers the use of ab194550 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 - 1/2500. Predicted molecular weight: 34 kDa.
ELISA Use at an assay dependent concentration.

Target

  • Function
    Somewhat implicated in the biogenesis of peroxisomes.
  • Involvement in disease
    Defects in PEX2 are the cause of peroxisome biogenesis disorder complementation group 5 (PBD-CG5) [MIM:170993]; also known as PBD-CGF. 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 PEX2 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 PEX2 are 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 pex2/pex10/pex12 family.
    Contains 1 RING-type zinc finger.
  • Cellular localization
    Peroxisome membrane.
  • Information by UniProt
  • Database links
  • Alternative names
    • 35 kDa peroxisomal membrane protein antibody
    • PAF-1 antibody
    • PAF1 antibody
    • Peroxin-2 antibody
    • peroxisomal biogenesis factor 2 antibody
    • Peroxisomal membrane protein 3 antibody
    • peroxisomal membrane protein 3, 35kDa antibody
    • Peroxisome assembly factor 1 antibody
    • Peroxisome biogenesis factor 2 antibody
    • PEX2 antibody
    • PEX2_HUMAN antibody
    • PMP3 antibody
    • PMP35 antibody
    • PXMP3 antibody
    • RING finger protein 72 antibody
    • RNF72 antibody
    • ZWS3 antibody
    see all

Images

  • Anti-PEX2 antibody - C-terminal (ab194550) at 1/1000 dilution + PEX2 human recombinant protein fragment at 0.2 µg

    Predicted band size: 34 kDa

  • Anti-PEX2 antibody - C-terminal (ab194550) at 1/500 dilution + HeLa cell lysate at 50 µg

    Predicted band size: 34 kDa

  • Anti-PEX2 antibody - C-terminal (ab194550) at 1/500 dilution + NIH3T3 cell lysate at 50 µg

    Predicted band size: 34 kDa

References

ab194550 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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