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Pyruvate kinase deficiency in children

Pediatric Blood & Cancer, 2021
AbstractBackgroundPyruvate kinase deficiency (PKD) is a rare, autosomal recessive red blood cell enzyme disorder, which leads to lifelong hemolytic anemia and associated complications from the disease and its management.MethodsAn international, multicenter registry enrolled 124 individuals younger than 18 years old with molecularly confirmed PKD from ...
Satheesh Chonat   +17 more
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Regulation of pyruvate kinase

Advances in Enzyme Regulation, 1969
Abstract The mechanism and sequence of events of the induction of liver pyruvate kinase by sucrose and fructose administration were examined. Actinomycin and cycloheximide prevented the sucrose and fructose induced rise in pyruvate kinase activity.
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Defective erythrocyte pyruvate kinase

Clinica Chimica Acta, 1976
A defective pyruvate kinase (EC 2.7.1.40) is described. The abnormal PK is characterized by a shift in the R in equilibrium T equilibrium to the T-state. The Ko.5 for the substrate phosphoenol pyruvate is about 6 times higher than for the normal enzyme, while the KM value for the positive effector Fru-1, 6-P2 is increased.
G E, Staal   +3 more
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Pyruvate kinase of Bacillus subtilis

Biochimica et Biophysica Acta (BBA) - Enzymology, 1972
Abstract Conditions are reported which stabilize pyruvate kinase (ATP: pyruvate phosphotransferase, EC 2.7.1.40) activity in extracts of Bacillus subtilis. These conditions include high protein concentrations and the presence of KCl and phosphoenolpyruvate.
M, Diesterhaft, E, Freese
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Relaxation spectra of pyruvate kinase

Biochimica et Biophysica Acta (BBA) - Enzymology, 1970
Relaxation studies using the temperature jump have been carried out on pyruvate kinase (ATP: pyruvate phosphotransferase, EC 2.7.1.40) isolated from rabbit muscle. Several concentration-independent relaxation effects with associated relaxation times of approx.
G G, Hammes, J, Simplicio
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Insulin: Inducer of Pyruvate Kinase

Science, 1965
The hepatic pyruvate kinase activity markedly decreased when rats were made diabetic by alloxan injection. Insulin treatment induced new synthesis of pyruvate kinase which was prevented by injection of ethionine and actinomycin D. The evidence indicated that the increased pyruvate kinase activity induced by insulin entails at a certain stage a ...
G, WEBER, N B, STAMM, E A, FISHER
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Pyruvate kinase of higher plants

Biochimica et Biophysica Acta (BBA) - General Subjects, 1973
Abstract 1. 1. Pyruvate kinase (EC 2.7.1.40) was extracted from pea seeds and carrots and partially purified by fractionation with (NH4)2SO4. 2. 2. The enzyme from both sources showed hyperbolic kinetics for the substrates phosphoenolpyruvate and ADP. Mg2+ or Mn2+ and a movement cation were required for enzyme activity.
J D, Tomlinson, J F, Turner
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Radiotracer assay of pyruvate kinase

Analytical Biochemistry, 1971
Abstract A radiotracer enzyme assay for the determination of pyruvate kinase activity is described. The rate of the enzyme catalyzed reaction is determined by radioassay of either ATP- 3 H formed or unreacted ADP- 3 H, or both after their separations from the assay reaction mixture by TLC.
J F, Morningstar, D G, Therriault
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Pyruvate Kinase Deficiency and Malaria

New England Journal of Medicine, 2008
Malaria that is caused by Plasmodium falciparum is a significant global health problem. Genetic characteristics of the host influence the severity of disease and the ultimate outcome of infection, and there is evidence of coevolution of the plasmodium parasite with its host.
Kodjo, Ayi   +7 more
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Turnover of liver pyruvate kinase

Archives of Biochemistry and Biophysics, 1980
Abstract The relative rates of synthesis and degradation for liver pyruvate kinase have been determined in rats fed standard lab chow, fasted, and refed a high-carbohydrate-low-protein diet. Relative rates of synthesis and apparent rates of degradation were determined by pulse-labeling the enzyme in vivo with l -[4,5- 3 H]leucine and by measuring ...
C, Cladaras, G L, Cottam
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