Results 121 to 130 of about 3,568 (156)
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Pyruvate carboxylase deficiency
Journal of Inherited Metabolic Disease, 1984AbstractThe causes of congenital lactic acidaemia are outlined. Isolated pyruvate carboxylase deficiency is reviewed in detail with a report of a recent case and a discussion of the biochemical consequences. Other causes of defective pyruvate carboxylation are described, particularly the combined carboxylase defects.
K, Bartlett +4 more
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Substrate activation of pyruvate carboxylase by pyruvate
Biochemical and Biophysical Research Communications, 1969Abstract A kinetic analysis of pyruvate carboxylase isolated from sheep liver and using pyruvate as the variable substrate revealed non-Michaelis Menten kinetic. Double reciprocal plots were biphasic and R S values of 222 were obtained. Hill plots prepared from the initial velocity data showed that at low pyruvate concentrations, the slope of the ...
H, Taylor, J, Nielsen, D B, Keech
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Clinical Biochemistry, 1990
Pyruvate kinase (PK) deficiency was initially described by Valentine et al. in 1961. Since then, more than 300 cases have been described, including 65 in Japan. PK deficiency is the most common hereditary nonspherocytic hemolytic anemia among several red cell enzyme defects of the Embden-Meyerhof glycolytic pathway.
S, Miwa, H, Fujii
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Pyruvate kinase (PK) deficiency was initially described by Valentine et al. in 1961. Since then, more than 300 cases have been described, including 65 in Japan. PK deficiency is the most common hereditary nonspherocytic hemolytic anemia among several red cell enzyme defects of the Embden-Meyerhof glycolytic pathway.
S, Miwa, H, Fujii
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Current Opinion in Anaesthesiology, 2008
Ethyl pyruvate is a simple derivative of the endogenous metabolite, pyruvic acid. Ethyl pyruvate was shown to ameliorate some of the structural and functional changes associated with mesenteric ischemia and reperfusion in rats.Treatment with ethyl pyruvate has been shown to improve survival and ameliorate organ dysfunction in a wide variety of ...
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Ethyl pyruvate is a simple derivative of the endogenous metabolite, pyruvic acid. Ethyl pyruvate was shown to ameliorate some of the structural and functional changes associated with mesenteric ischemia and reperfusion in rats.Treatment with ethyl pyruvate has been shown to improve survival and ameliorate organ dysfunction in a wide variety of ...
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Transpulmonary pyruvate kinetics
American Journal of Physiology-Regulatory, Integrative and Comparative Physiology, 2011Shuttling of intermediary metabolites, such as pyruvate, contributes to the dynamic energy and biosynthetic needs of tissues. Tracer kinetic studies offer a powerful tool to measure the metabolism of substrates like pyruvate that are simultaneously taken up from and released into the circulation by organs.
Matthew L, Johnson +3 more
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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
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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
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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.
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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
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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
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