Urea as a Selective Inhibitor of Argininosuccinate Lyase

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Nitro analogs of substrates for argininosuccinate synthetase and argininosuccinate lyase.

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Kinetic mechanism of bovine liver argininosuccinate lyase.

The kinetic mechanism of bovine liver argininosuccinate lyase has been determined at pH 7.5, 25 degrees C. Fumarate and arginine are both noncompetitive inhibitors versus argininosuccinate. The dead-end inhibitor, succinate, is competitive versus fumarate and argininosuccinate, but noncompetitive versus arginine. Citrulline is competitive versus arginine and noncompetitive versus argininosuccin...

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Isotopic probes of the argininosuccinate lyase reaction.

The mechanism of the argininosuccinate lyase reaction has been probed by the measurement of the effects of isotopic substitution at the reaction centers. A primary deuterium isotope effect of 1.0 on both V and V/K is obtained with (2S,3R)-argininosuccinate-3-d, while a primary 15N isotope effect on V/K of 0.9964 +/- 0.0003 is observed. The 15N isotope effect on the equilibrium constant is 1.018...

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A positional isotope exchange study of the argininosuccinate lyase reaction.

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Refeeding syndrome in a young woman with argininosuccinate lyase deficiency☆

A severely chronically protein and calorie restricted young woman with argininosuccinate lyase deficiency developed transient refeeding syndrome (RFS) and hyperammonemia after modest diet liberalization following initiation of glycerol phenylbutyrate (GPB). The patient required IV supportive care and supplementation with potassium, magnesium and calcium. She is now doing well on GPB and an appr...

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ژورنال

عنوان ژورنال: European Journal of Biochemistry

سال: 1977

ISSN: 0014-2956,1432-1033

DOI: 10.1111/j.1432-1033.1977.tb11541.x