Results 211 to 220 of about 27,529 (245)

Pyruvate Carboxylase Deficiency

1995
Pyruvate carboxylase (PC) deficiency is a rare disorder with autosomal recessive inheritance. Generally, two clinical and biochemical phenotypes of isolated PC deficiency can be distinguished. The so-called French phenotype presents in the neonatal period with severe lactic acidemia and is clinically characterized by failure to thrive, anorexia ...
Marjo S. van der Knaap, Jacob Valk
openaire   +1 more source

Pyruvate Carboxylase and Pyruvate Dehydrogenase Deficiency

2014
Pyruvate carboxylase and pyruvate dehydrogenase deficiency are the most common disorders in pyruvate metabolism and almost always affect the central nervous system. The severity and the clinical phenotypes vary, with a range from overwhelming neonatal lactic acidosis and early death to milder presentations.
openaire   +1 more source

Assays of Pyruvate Dehydrogenase Complex and Pyruvate Carboxylase Activity

2011
Pyruvate dehydrogenase complex (PDC) and pyruvate carboxylase (PC) are mitochondrial enzymes that provide the initial steps of the two main alternatives for pyruvate metabolism: oxidative decarboxylation vs. anaplerotic carboxylation, gluconeogenesis, and glycerogenesis.
Douglas, Kerr, George, Grahame, Ghunwa, Nakouzi   +2 more
openaire   +2 more sources

Pyruvate Carboxylase

1984
P V, Attwood, D B, Keech
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Pyruvate carboxylase activity in lymphoblasts

Journal of Inherited Metabolic Disease, 1982
G, Takada, M, Ohtake, S, Miyabayashi, K, Narisawa, K, Tada   +4 more
openaire   +2 more sources

1 Pyruvate Carboxylase

1972
Publisher Summary This chapter discusses the properties and structure of pyruvate carboxylase. Pyruvate carboxylase activity has been detected in most mammalian tissues examined, although high maximal catalytic capacities for this enzyme are confined to the liver, kidney cortex, and adipose tissue.
openaire   +1 more source

Glucose metabolism and pyruvate carboxylase enhance glutathione synthesis and restrict oxidative stress in pancreatic islets

Cell Reports, 2021
Accalia Fu, Lara Van Rooyen, Daina Avizonis   +2 more
exaly  

Pyruvate carboxylase

2021
openaire   +1 more source

Pyruvate-Carboxylase-Mediated Anaplerosis Promotes Antioxidant Capacity by Sustaining TCA Cycle and Redox Metabolism in Liver

Cell Metabolism, 2019
Stanislaw Deja, Blanka Kucejová, Justin A Fletcher   +2 more
exaly  

Pyruvate Carboxylase Activity in Primary Cultures of Astrocytes and Neurons

Journal of Neurochemistry, 1983
J Drejer, L Hertz, Arne Schousboe
exaly