製品の概要

製品の詳細

  • 由来
    Synthetic
  • アミノ酸配列
    • 生物種
      Human
    • 配列
      KPWKPGDKEPCAH
    • 領域
      407 to 419

関連製品

特性

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.

  • アプリケーション

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

  • 製品の状態
    Liquid
  • Concentration information loading...

前処理および保存

  • 保存方法および安定性

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

関連情報

  • 別名
    • 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
  • 機能
    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.
  • パスウェイ
    Carbohydrate biosynthesis; gluconeogenesis.
  • 関連疾患
    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.
  • 配列類似性
    Belongs to the phosphoenolpyruvate carboxykinase [GTP] family.
  • 翻訳後修飾
    Phosphorylated upon DNA damage, probably by ATM or ATR.
  • 細胞内局在
    Mitochondrion.
  • Information by UniProt

参考文献

ab45819 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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