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Phosphoenolpyruvate carboxykinase deficiency

Known as: Phosphoenolpyruvate Carboxykinase (GTP) Deficiency, PCK1 DEFICIENCY, CYTOSOLIC, Phosphopyruvate carboxylase deficiency 
A very rare, autosomal recessive inherited disorder caused by deficiency of the enzyme phosphoenolpyruvate carboxykinase, which is involved in… 
National Institutes of Health

Related topics

Related topics

12 relations
Autosomal recessive inheritanceCerebral atrophyCyanosisEnzyme Deficiency

Broader (2)

GTP-Dependent Phosphoenolpyruvate CarboxykinaseLiver diseases

Papers overview

Semantic Scholar uses AI to extract papers important to this topic.
1992
1992
Gluconeogenesis in hepatocytes isolated from the skipjack tuna (Katsuwonus pelamis)
Skipjack tuna (Katsuwonus pelamis) hepatocytes were isolated and determined to be viable based on trypan blue exclusion, energy… 
1986
1986
Stereochemical course of thiophosphoryl transfer catalyzed by cytosolic phosphoenolpyruvate carboxykinase.
Rat liver cytosolic phosphoenolpyruvate carboxykinase (PEPCK) utilizes inosine 5'-(3-thiotriphosphate) (ITP gamma S) as an… 
1986
1986
Studies on the early changes in rat hepatic fructose 2,6-bisphosphate and enzymes in response to a high protein diet.
Pertinent hepatic metabolites and enzymes were examined in rats fed a high carbohydrate (HC) diet and during the first 24 h of… 
1984
1984
Stereoselectivity of interaction of phosphoenolpyruvate analogues with various phosphoenolpyruvate-utilizing enzymes.
The halogenated phosphoenolpyruvate analogues (Z)-phosphoenol-3-fluoropyruvate, (E)-phosphoenol-3-fluoropyruvate, and (Z… 
1983
1983
Induction of phosphoenolpyruvate carboxykinase by hydrocortisone in rat liver and brain as a function of age.
The activity and induction pattern of phosphoenolpyruvate carboxykinase (PEPCK) in the liver and brain of young (6-), adult (30… 
1980
1980
Intracellular distribution of carbon dioxide-fixing enzymes in Trypanosoma cruzi and Crithidia fasciculata.
The intracellular distribution of phosphoenopyruvate carboxykinase (EC 4.1.1.49) and NADP-linked malic enzyme (EC 1.1.1.40… 
1973
1973
Hypoglycemia associated with lipid accumulation in primary hepatocellular carcinoma
A 54‐year‐old Chinese patient with a slowly growing hepatocellular carcinoma developed persistent hypoglycemia and received… 
1972
1972
Regulation by phosphoenolpyruvate of fructose-1,6-diphosphatase in skeletal muscle: evidence for an allosteric activator of the enzyme.
Fructose-1,6-diphosphatase (FDPase) from muscle of three cold-water crustaceans was found to be activated by phosphoenolpyruvate… 
Highly Cited
1970
Highly Cited
1970
Regulation of gluconeogenesis in the guinea pig liver.
Because of differences in the pattern of enzyme activities involved in glycolysis and gluconeo-genesis between rat and guinea pig… 
Highly Cited
1969
Highly Cited
1969
Intracellular localization of pyruvate carboxylase and phosphoenolpyruvate carboxykinase in rat liver.
The overall activity and distribution of pyruvate carboxylase and of phosphoenolpyruvate carboxykinase in rat liver was studied…