Recombinant Human XPNPEP3 protein (ab173067)

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Description

  • Product name

    Recombinant Human XPNPEP3 protein

  • Purity

    > 95 % SDS-PAGE.
    ab173067 is greater than 95% pure, as determined by SEC-HPLC and reducing SDS-PAGE.

  • Endotoxin level

    < 1.000 Eu/µg

  • Expression system

    Escherichia coli

  • Accession

    Q9NQH7

  • Protein length

    Full length protein

  • Animal free

    No

  • Nature

    Recombinant

    • Species

      Human

    • Sequence

      MGSSHHHHHHSSGLVPRGSHMPWLLSAPKLVPAVANVRGLSGCMLCSQRR YSLQPVPERRIPNRYLGQPSPFTHPHLLRPGEVTPGLSQVEYALRRHKLM SLIQKEAQGQSGTDQTVVVLSNPTYYMSNDIPYTFHQDNNFLYLCGFQEP DSILVLQSLPGKQLPSHKAILFVPRRDPSRELWDGPRSGTDGAIALTGVD EAYTLEEFQHLLPKMKAETNMVWYDWMRPSHAQLHSDYMQPLTEAKAKSK NKVRGVQQLIQRLRLIKSPAEIERMQIAGKLTSQAFIETMFTSKAPVEEA FLYAKFEFECRARGADILAYPPVVAGGNRSNTLHYVKNNQLIKDGEMVLL DGGCESSCYVSDITRTWPVNGRFTAPQAELYEAVLEIQRDCLALCFPGTS LENIYSMMLTLIGQKLKDLGIMKNIKENNAFKAARKYCPHHVGHYLGMDV HDTPDMPRSLPLQPGMVITIEPGIYIPEDDKDAPEKFRGLGVRIEDDVVV TQDSPFILSADCPKEMNDIEQICSQASLEHHHHHH

    • Predicted molecular weight

      60 kDa including tags

    • Amino acids

      1 to 507

    • Tags

      His tag C-Terminus , His tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab173067 in the following tested applications.

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

  • Applications

    HPLC

    SDS-PAGE

  • Form

    Liquid
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped on Dry Ice. Store at -20°C or -80°C. Avoid freeze / thaw cycle.

    pH: 7.30
    Constituents: 0.02% DTT, 0.3% Tris

    It is supplied as an 0.2 µM filtered solution.

General Info

  • Alternative names

    • Aminopeptidase P3
    • APP3
    • NPHPL1
    • OTTHUMP00000199806
    • Probable Xaa Pro aminopeptidase 3
    • Probable Xaa-Pro aminopeptidase 3
    • X Pro aminopeptidase 3
    • X prolyl aminopeptidase (aminopeptidase P) 3
    • X-Pro aminopeptidase 3
    • XPNPEP3
    • XPP3_HUMAN
    see all

  • Tissue specificity

    Isoform 1 and isoform 2 are widely expressed, with isoform 1 being more abundant.

  • Involvement in disease

    Defects in XPNPEP3 are the cause of nephronophthisis-like nephropathy type 1 (NPHPL1) [MIM:613159]. A disorder with features of nephronophthisis, a cystic kidney disease leading to end-stage renal failure. Nephronophthisis is histologically characterized by modifications of the tubules with thickening of the basement membrane, interstitial fibrosis and, in the advanced stages, medullary cysts. Typical clinical manifestation are chronic renal failure, anemia, polyuria, polydipsia, isosthenuria, and growth retardation. Associations with extrarenal symptoms are frequent. In NPHPL1 patients, extrarenal symptoms include hypertension, essential tremor, sensorineural hearing loss and gout. Severely affected individuals can manifest a mitochondrial disorder with isolated complex I deficiency activity in muscle, seizures, mental retardation and hypertrophic dilated cardiomyopathy.

  • Sequence similarities

    Belongs to the peptidase M24B family.

  • Cellular localization

    Mitochondrion.
  • Information by UniProt

References

ab173067 has not yet been referenced specifically in any publications.

Publishing research using ab173067? Please let us know so that we can cite the reference in this datasheet.

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