Overview

  • Product name
    Recombinant Human PEX5 protein
  • Protein length
    Full length protein

Description

  • Nature
    Recombinant
  • Source
    Wheat germ
  • Amino Acid Sequence
    • Species
      Human
    • Sequence
      MAMRELVEAECGGANPLMKLAGHFTQDKALRQEGLRPGPWPPGAPASEAA SKPLGVASEDELVAEFLQDQNAPLVSRAPQTFKMDDLLAEMQQIEQSNFR QAPQRAPGVADLALSENWAQEFLAAGDAVDVTQDYNETDWSQEFISEVTD PLSVSPARWAEEYLEQSEEKLWLGEPEGTATDRWYDEYHPEEDLQHTASD FVAKVDDPKLANSEFLKFVRQIGEGQVSLESGAGSGRAQAEQWAAEFIQQ QGTSDAWVDQFTRPVNTSALDMEFERAKSAIELQAELEEMAKRDAEAHPW LSDYDDLTSATYDKGYQFEEENPLRDHPQPFEEGLRRLQEGDLPNAVLLF EAAVQQDPKHMEAWQYLGTTQAENEQELLAISALRRCLELKPDNQTALMA LAVSFTNESLQRQACETLRDWLRYTPAYAHLVTPAEEGAGGAGLGPSKRI LGSLLSDSLFLEVKELFLAAVRLDPTSIDPDVQCGLGVLFNLSGEYDKAV DCFTAALSVRPNDYLLWNKLGATLANGNQSEEAVAAYRRALELQPGYIRS RYNLGISCINLGAHREAVEHFLEALNMQRKSRGPRGEGGAMSENIWSTLR LALSMLGQSDAYGAADARDLSTLLTMFGLPQ
    • Amino acids
      1 to 631
    • Tags
      proprietary tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab159293 in the following tested applications.

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

  • Applications

    ELISA

    Western blot

  • Form
    Liquid
  • Additional notes
    Protein concentration is above or equal to 0.05 mg/ml.
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.31% Glutathione, 0.79% Tris HCl

General Info

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

Images

  • ab159293 on a 12.5% SDS-PAGE stained with Coomassie Blue.

References

ab159293 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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