Recombinant Human XPNPEP3 protein (ab163756)

Overview

Description

  • Nature
    Recombinant
  • Source
    Wheat germ
  • Amino Acid Sequence
    • Species
      Human
    • Sequence
      MPWLLSAPKLVPAVANVRGLSGCMLCSQRRYSLQPVPERRIPNRYLGQPS PFTHPHLLRPGEVTPGLSQVEYALRRHKLMSLIQKEAQGQSGTDQTVVVL SNPTYYMSNDIPYTFHQDNNFLYLCGFQEPDSILVLQSLPGKQLPSHKAI LFVPRRDPSRELWDGPRSGTDGAIALTGVDEAYTLEEFQHLLPKMKAETN MVWYDWMRPSHAQLHSDYMQPLTEAKAKSKNKVRGVQQLIQRLRLIKSPA EIERMQIAGKLTSQAFIETMFTSKAPVEEAFLYAKFEFECRARGADILAY PPVVAGGNRSNTLHYVKNNQLIKDGEMVLLDGGCESSCYVSDITRTWPVN GRFTAPQAELYEAVLEIQRDCLALCFPGTSLENIYSMMLTLIGQKLKDLG IMKNIKENNAFKAARKYCPHHVGHYLGMDVHDTPDMPRSLPLQPGMVITI EPGIYIPEDDKDAPEKFRGLGVRIEDDVVVTQDSPLILSADCPKEMNDIE QICSQAS
    • Amino acids
      1 to 507
    • Tags
      proprietary tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab163756 in the following tested applications.

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

  • Applications

    Western blot

    ELISA

  • 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
    • 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

Images

  • ab163756 on a 12.5% SDS-PAGE stained with Coomassie Blue.

References

ab163756 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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