Overview

Description

  • NatureSynthetic
  • Amino Acid Sequence
    • SpeciesHuman
    • SequenceKPWKPGDKEPCAH
    • Amino acids407 to 419

Associated products

Specifications

Our Abpromise guarantee covers the use of ab45819 in the following tested applications.

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

  • Applications

    Blocking - Blocking peptide for Anti-PCK2 antibody (ab40843)

  • FormLiquid
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.

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
  • FunctionCatalyzes 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.
  • PathwayCarbohydrate biosynthesis; gluconeogenesis.
  • Involvement in diseaseDefects 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 similaritiesBelongs to the phosphoenolpyruvate carboxykinase [GTP] family.
  • Post-translational
    modifications
    Phosphorylated upon DNA damage, probably by ATM or ATR.
  • Cellular localizationMitochondrion.
  • Information by UniProt

References for PCK2 peptide (407-419) (ab45819)

ab45819 has not yet been referenced specifically in any publications.

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