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Phosphoenolpyruvate (PEP)

MBS653912

1 g

525 USD

Substrate

Phosphoenolpyruvate (PEP)

[Phosphoenolpyruvate]

[phosphoenolpyruvate carboxykinase; Phosphoenolpyruvate carboxykinase [GTP], mitochondrial; phosphoenolpyruvate carboxykinase [GTP], mitochondrial; phosphoenolpyruvate carboxykinase [GTP], mitochondrial; PEP carboxykinase; phosphopyruvate carboxylase; phosphoenolpyruvate carboxylase; phosphoenolpyruvate carboxykinase 2 (mitochondrial); Phosphoenolpyruvate carboxylase]

[PCK2; PCK2; PEPCK; PEPCK2; PEPCK-M; PEPCK2; PEPCK-M]

PCKGM_HUMAN

Highly Purified. 98.6%

Lyophilized powder may be stored at -20 degree C. Stable for 12 months at -20 degree C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Testing Data

Molecular Formula: C3H4O6P C6H14N. Water: 0.1%. Pyruvate content: 0.005%

Molecular Biology

66346721

NP_004554.2

NM_004563.2

Q16822

267.22 anhy

Adipocytokine Signaling Pathway (83093); Adipocytokine Signaling Pathway (505); Adipogenesis Pathway (198832); Citrate Cycle (TCA Cycle) Pathway (82927); Citrate Cycle (TCA Cycle) Pathway (288); DNA Damage Response (only ATM Dependent) Pathway (198827); Glucocorticoid Receptor Regulatory Network Pathway (138014); Gluconeogenesis Pathway (106204); Gluconeogenesis, Oxaloacetate = Fructose-6P Pathway (413342); Gluconeogenesis, Oxaloacetate = Fructose-6P Pathway (468196)

This gene encodes a member of the phosphoenolpyruvate carboxykinase (GTP) family. The protein is a mitochondrial enzyme that catalyzes the conversion of oxaloacetate to phosphoenolpyruvate in the presence of GTP. A cytosolic form encoded by a different gene has also been characterized and is the key enzyme of gluconeogenesis in the liver. The encoded protein may serve a similar function, although it is constitutively expressed and not modulated by hormones such as glucagon and insulin that regulate the cytosolic form. Alternatively spliced transcript variants have been described. [provided by RefSeq, Jul 2008]

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 . By similarity. Catalytic activity: GTP + oxaloacetate = GDP + phosphoenolpyruvate + CO2. Cofactor: Manganese . By similarity.Binds 1 manganese ion per subunit . By similarity. Pathway: Carbohydrate biosynthesis; gluconeogenesis. Subunit structure: Monomer. Subcellular location: Mitochondrion. Involvement in disease: Mitochondrial phosphoenolpyruvate carboxykinase deficiency (M-PEPCKD) [MIM:261650]: 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.Note: The disease is caused by mutations affecting the gene represented in this entry. Miscellaneous: In eukaryotes there are two isozymes: a cytoplasmic one and a mitochondrial one. Sequence similarities: Belongs to the phosphoenolpyruvate carboxykinase [GTP] family. Sequence caution: The sequence CAD62600.1 differs from that shown. Reason: Erroneous initiation.

1 g

525 USD