Human PCK2 peptide (ab87120)
- Product nameHuman PCK2 peptideSee all PCK2 proteins and peptides ...
Our Abpromise guarantee covers the use of ab87120 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
- Purity70 - 90% by HPLC.
- Concentration information loading...
Preparation and Storage
- Stability and Storage
Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
Information available upon request.
- EC 220.127.116.11
- GTP mitochondrial precursor
- Mitochondrial phosphoenolpyruvate carboxykinase 2
- PEP carboxykinase
- PEPCK deficiency mitochondrial
- PEPCK M
- Phosphoenolpyruvate carboxykinase [GTP]
- Phosphoenolpyruvate carboxykinase 2 (mitochondrial)
- Phosphoenolpyruvate carboxykinase 2 mitochondrial
- Phosphoenolpyruvate carboxylase
- Phosphopyruvate carboxylase
- 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.
modificationsPhosphorylated upon DNA damage, probably by ATM or ATR.
- Cellular localizationMitochondrion.
References for Human PCK2 peptide (ab87120)
ab87120 has not yet been referenced specifically in any publications.