Overview

  • Product name

    Recombinant Human PCK2 protein
  • Protein length

    Full length protein

Description

  • Nature

    Recombinant
  • Source

    Wheat germ
  • Amino Acid Sequence
    • Accession
    • Species

      Human
    • Sequence

      MAALYRPGLRLNWHGLSPLGWPSCRSIQTLRVLSGDLGQLPTGIRDFVEH SARLCQPEGIHICDGTEAENTATLTLLEQQGLIRKLPKYNNCWLARTDPK DVARVESKTVIVTPSQRDTVPLPPGGARGQLGNWMSPADFQRAVDERFPG CMQGRTMYVLPFSMGPVGSPLSRIGVQLTDSAYVVASMRIMTRLGTPVLQ ALGDGDFVKCLHSVGQPLTGQGEPVSQWPCNPEKTLIGHVPDQREIISFG SGYGGNSLLGKKCFALRIASRLARDEGWLAEHMLILGITSPAGKKRYVAA AFPSACGKTNLAMMRPALPGWKVECVGDDIAWMRFDSEGRLRAINPENGF FGVAPGTSATTNPNAMATIQSNTIFTNVAETSDGGVYWEGIDQPLPPGVT VTSWLGKPWKPGDKEPCAHPNSRFCAPARQCPIMDPAWEAPEGVPIDAII FGGRRPKGVPLVYEAFNWRHGVFVGSAMRSESTAAAEHKGKIIMHDPFAM RPFFGYNFGHYLEHWLSMEGRKGAQLPRIFHVNWFRRDEAGHFLWPGFGE NARVLDWICRRLEGEDSARETPIGLVPKEGALDLSGLRAIDTTQLFSLPK DFWEQEVRDIRSYLTEQVNQDLPKEVLAELEALERRVHKM
    • Molecular weight

      96 kDa
    • Amino acids

      1 to 640

Specifications

Our Abpromise guarantee covers the use of ab132473 in the following tested applications.

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

  • Applications

    ELISA

    SDS-PAGE

    Western blot

  • Form

    Liquid
  • Additional notes

    Protein concentration is above or equal to 0.05 µg/µl.
    Best use within three months from the date of receipt of this protein.
  • 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

    • EC 4.1.1.32
    • GTP mitochondrial precursor
    • HGNC:8725
    • mitochondrial
    • Mitochondrial phosphoenolpyruvate carboxykinase 2
    • OTTHUMP00000164700
    • PCK2
    • PCKGM_HUMAN
    • PE
    • PEP carboxykinase
    • PEPCK
    • PEPCK deficiency mitochondrial
    • PEPCK M
    • PEPCK-M
    • PEPCK2
    • Phosphoenolpyruvate carboxykinase [GTP]
    • Phosphoenolpyruvate carboxykinase 2 (mitochondrial)
    • Phosphoenolpyruvate carboxykinase 2 mitochondrial
    • Phosphoenolpyruvate carboxylase
    • Phosphopyruvate carboxylase
    see all
  • Function

    Catalyzes the conversion of oxaloacetate (OAA) to phosphoenolpyruvate (PEP), the rate-limiting step in the metabolic pathway that produces glucose from lactate and other precursors derived from the citric acid cycle.
  • Pathway

    Carbohydrate biosynthesis; gluconeogenesis.
  • Involvement in disease

    Defects in PCK2 are the cause of mitochondrial phosphoenolpyruvate carboxykinase deficiency (M-PEPCKD) [MIM:261650]. A metabolic disorder resulting from impaired gluconeogenesis. It is a rare disease with less than 10 cases reported in the literature. Clinical characteristics include hypotonia, hepatomegaly, failure to thrive, lactic acidosis and hypoglycemia. Autoposy reveals fatty infiltration of both the liver and kidneys. The disorder is transmitted as an autosomal recessive trait.
  • Sequence similarities

    Belongs to the phosphoenolpyruvate carboxykinase [GTP] family.
  • Post-translational
    modifications

    Phosphorylated upon DNA damage, probably by ATM or ATR.
  • Cellular localization

    Mitochondrion.
  • Information by UniProt

Images

  • SDS-PAGE analysis of ab132473 on a 12.5% gel stained with Coomassie Blue.

References

ab132473 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

There are currently no Customer reviews or Questions for ab132473.
Please use the links above to contact us or submit feedback about this product.

Please note: All products are "FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC PROCEDURES"
For licensing inquiries, please contact partnerships@abcam.com

Sign up