Synthetic peptide corresponding to Human PCK2 (internal region).
Sequence:
KPWKPGDKEPCAH
Host:
Goat
Clonality:
Polyclonal
Isotype:
IgG
Conjugate:
Unconjugated
Purification:
Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.
Concentration:
100 μg at 0.5 mg/ml.
Product Form:
Liquid
Formulation:
Supplied in Tris Buffered Saline, pH 7.30, with 0.02% Sodium Azide and 0.5% BSA.
Storage:
Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
Target
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.
Involvement in Disease:
Mitochondrial phosphoenolpyruvate carboxykinase deficiency: 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. Autopsy 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.