概述

描述

  • 性质
    Synthetic
  • 氨基酸序列
    • 种属
      Human
    • 序列
      KPWKPGDKEPCAH
    • 氨基酸
      407 to 419

相关产品

技术指标

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.

  • 应用

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

  • 形式
    Liquid
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

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

常规信息

  • 别名
    • 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
  • 功能
    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.
  • 通路
    Carbohydrate biosynthesis; gluconeogenesis.
  • 疾病相关
    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.
  • 序列相似性
    Belongs to the phosphoenolpyruvate carboxykinase [GTP] family.
  • 翻译后修饰
    Phosphorylated upon DNA damage, probably by ATM or ATR.
  • 细胞定位
    Mitochondrion.
  • Information by UniProt

文献

ab45819 has not yet been referenced specifically in any publications.

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