Results 161 to 170 of about 3,470 (203)

Stabilization effects of kosmotrope systems on ornithine carbamoyltransferase

International Journal of Biological Macromolecules, 2009
In the present article the influence of salts and additives, such as trehalose, NaCl, ornithine, sodium phosphate and ammonium sulphate, on ornithine carbamoyltransferase (OCTase) is investigated in order to study the OCTase stabilization process as a function of solutes and to point out the fundamental role played by an enhancement of hydrophobic ...
BARRECA, Davide, BELLOCCO, Ersilia Santa, GALLI, Giovanni, LAGANA', Giuseppina, LEUZZI, Ugo, MAGAZU', Salvatore, MIGLIARDO, Federica, GALTIERI, Antonio, M. T. Telling   +8 more
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Affinity of arginase for ornithine carbamoyltransferase in Saccharomyces cerevisiae

Journal of Molecular Biology, 1976
Abstract The association between the two trimeric enzymes ornithine carbamoyltransferase and arginase, which is under the control of arginine and ornithine, is endothermic (Δ H ° = + 14.6 kcal mol −1 ). The process is clearly entropy-driven (Δ S ° = + 94.7 cal mol −1 deg.
Penninckx, Michel, Wiame, Jean-Marie
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Ornithine carbamoyltransferase deficiency with subnormal enzyme activity

Journal of Inherited Metabolic Disease, 1991
Ornithine carbamoyltransferase (OCT; EC 2.1.3.3) deficiency, an X-linked disorder (McKusick 31125) presents many forms of onset in male patients (Walser, 1983) with residual hepatic activity for OCT ranging from zero to subnormal values (Briand et al., 1982; Rabier et al., 1989).
D, Rabier, B, Guillois, J, Bardet, C, Deprun, P, Parvy, P, Kamoun   +5 more
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Ornithine carbamoyltransferase deficiency presenting with chorea in a female

Journal of Inherited Metabolic Disease, 2000
De¢ciency of ornithine carbamoyltransferase (OCT, EC 2.1.3.3; McKusick 311250) is an X-linked disorder of the urea cycle. Symptomatic carriers present with a wide range of symptoms due to hyperammonaemic encephalopathy (Brusilow and Horwich 1995). We present a case of OCT de¢ciency in an 11-month-old female with the unusual feature of transient chorea ...
Wiltshire, E., Poplawski, N., Harbord, M., Harrison, R., Fletcher, J.   +4 more
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Anabolic Ornithine Carbamoyltransferase of Escherichia coli and Catabolic Ornithine Carbamoyltransferase of Pseudomonas putida. Steady-State Kinetic Analysis

European Journal of Biochemistry, 1978
The anabolic and catabolic ornithine carbamoyltransferases of Pseudomonas putida display an undirectional catalytic specialization: in citrulline synthesis for the anabolic enzyme, in citrulline phosphorolysis for the catabolic one. The irreversibility of the anabolic enzyme in vitro has been previously explained by its kinetic properties, whereas the ...
Wargnies, Brigitte, Legrain, Christiane, Stalon, Victor   +2 more
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A novel method for measuring serum ornithine carbamoyltransferase

Annals of Clinical Biochemistry: International Journal of Laboratory Medicine, 2003
Background: Serum ornithine carbamoyltransferase is a diagnostic marker of hepatic disorders due to its localization in periportal mitochondria. Methods: We have developed a new method for the determination of serum ornithine carbamoyltransferase.
Hiroaki, Ishikawa, Takeo, Matsuzawa, Koji, Ohashi, Yoichi, Nagamura   +3 more
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An Essential Lysine in the Substrate‐Binding Site of Ornithine Carbamoyltransferase

European Journal of Biochemistry, 1996
Treatment of ornithine carbamoyltransferase from dolphin Stenella with pyridoxal phosphate, followed by reduction with NaBH4, resulted in complete loss of enzyme activity. The phosphate alone or the substrate analogue 2‐aminovaleric acid moderately decreased the extent of inactivation, while carbamoyl phosphate plus 2‐aminovaleric acid provided ...
VALENTINI G, DE GREGORIO, Ambra, DISALVO C, GRIMM R, BELLOCCO, Ersilia Santa, CUZZOCREA G, IADAROLA P.   +6 more
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Ornithine carbamoyltransferase deficiency: molecular characterization of 29 families

Clinical Genetics, 2013
Ornithine carbamoyltransferase deficiency is the most common inherited defect of the urea cycle. We examined 28 male and 9 female patients from 29 families and identified 25 distinct mutations in OTC, 14 of which were novel. Three novel missense mutations (p.Ala102Pro, p.Pro158Ser, p.Lys210Glu) and a novel deletion of the Leu43 are not directly ...
G, Storkanova, H, Vlaskova, N, Chuzhanova, J, Zeman, V, Stranecky, F, Majer, K, Peskova, O, Luksan, M, Jirsa, M, Hrebicek, L, Dvorakova   +10 more
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Ornithine Carbamoyltransferase in Liver of the Dipnoan Protopterus aethiopicus

Science, 1965
Ornithine carbamoyltransferase, an enzyme that occurs typically in vertebrates having a ureotelic metabolism, is present in the liver of the African lungfish Protopterus aethiopicus . The reaction studied depends on L-ornithine, carbamoylphosphate, and unboiled homogenate of liver.
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Measurement of tissue ornithine carbamoyltransferase

Biochimica et Biophysica Acta (BBA) - Specialized Section on Enzymological Subjects, 1964
W F, VINCENT, J A, CAMERON
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