Results 81 to 90 of about 2,569 (111)
Some of the next articles are maybe not open access.
Amperometric sensor for pyruvate with immobilized pyruvate oxidase
Analytica Chimica Acta, 1987Abstract Several procedures for immobilization of pyruvate oxidase by chemical bonding are reported. Attachment to nylon net was unsatisfactory in terms of yield and stability. Polyazetidine, a new commercially available prepolymer and a new nylon membrane with surface carboxyl groups provided good long-term stability, up to 30 days in the case of ...
M. Mascini, MAZZEI, Franco
openaire +1 more source
Pyruvate carboxylase: Affinity labelling of the pyruvate binding site
Biochemical and Biophysical Research Communications, 1975Abstract The active-site-directed reagent, bromopyruvate has been used to covalently label the pyruvate binding site of pyruvate carboxylase (E.C.6.4.1.1.) isolated from sheep liver. Oxalo-acetate proved to be the most effective reaction component in protecting the enzyme against inactivation; pyruvate was less effective although its efficiency was ...
P J, Hudson, D B, Keech, J C, Wallace
openaire +2 more sources
Pyruvate kinase activators for treatment of pyruvate kinase deficiency
Hematology, 2023Abstract Pyruvate kinase (PK) deficiency is a congenital hemolytic anemia with wide-ranging clinical symptoms and complications associated with significant morbidity and reduced health-related quality of life in both children and adults.
openaire +2 more sources
Pyruvate Metabolism and Control: Factors Affecting Pyruvic Carboxylase Activity
Science, 1964Pyruvic carboxylase activated by acetyl coenzyme A is highly active in the mitochondria of rodent liver, and its activity is increased in fasting and alloxan diabetes. In conjunction with acyl carboxylase activated by di- and tricarboxylic acid, it forms a reciprocating control network.
A D, FREEDMAN, L, KOHN
openaire +2 more sources
Disorders of pyruvate carboxylase and the pyruvate dehydrogenase complex
Journal of Inherited Metabolic Disease, 1996SummaryThe most common defect associated with deficiency of the pyruvate dehydrogenase (PDH) complex occurs in the E1 component, specifically due to mutations in the X‐linked E1α gene. Clinical sequelae of these mutations, which range from severe neonatal lactic acidosis to carbohydrate‐sensitive ataxia, can be different in males and females depending ...
B H, Robinson +3 more
openaire +2 more sources
Assays of Pyruvate Dehydrogenase Complex and Pyruvate Carboxylase Activity
2011Pyruvate 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 +2 more
openaire +2 more sources
Pyruvate Carboxylase and Pyruvate Dehydrogenase Deficiency
2014Pyruvate 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
Nihon rinsho. Japanese journal of clinical medicine, 1974
PK deficiency is the most common and well characterized erythroenzymopathy in the Embden-Meyerhof glycolytic pathway associated with hereditary hemolytic anemia. History of discovery, clinical features, biochemical studies, as well as recent studies on molecular level were reviewed, with a stress on our own studies. As reference materials, Tables 2 and
openaire +3 more sources
PK deficiency is the most common and well characterized erythroenzymopathy in the Embden-Meyerhof glycolytic pathway associated with hereditary hemolytic anemia. History of discovery, clinical features, biochemical studies, as well as recent studies on molecular level were reviewed, with a stress on our own studies. As reference materials, Tables 2 and
openaire +3 more sources