Overview

Description

  • Nature
    Synthetic
  • Amino Acid Sequence
    • Species
      Human
    • Sequence
      KPWKPGDKEPCAH
    • Amino acids
      407 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)

  • Form
    Liquid
  • 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
  • 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

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

ab45819 has not yet been referenced specifically in any publications.

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