Results 21 to 30 of about 4,935 (147)

The Urea Carboxylase and Allophanate Hydrolase Activities of Urea Amidolyase Are Functionally Independent [PDF]

, 2016
Urea amidolyase (UAL) is a multifunctional biotin-dependent enzyme that contributes to both bacterial and fungal pathogenicity by catalyzing the ATP-dependent cleavage of urea into ammonia and CO2.
Bahler, Baker, Balotra, Bratlie, Chapman-Smith, Chen, Chen, Cheng, Dumetz, Fan, Fan, Geck, Ghosh, Greenberg, Hettwer, Huang, Ianeselli, James, Kaessmann, Kanamori, Kanamori, Khosla, Kimura, Koonin, Kotlyar, Kozer, Kulkarni, Kulkarni, Kuriyan, Lawrence, Lherbet, Lietzan, Lin, Lorenz, Marsh, Moxley, Navarathna, Navarathna, Ribeiro, Shapir, Shapir, Singh, Spivey, Strope, Thanabalu, Thoden, Thompson, Whitney, Zeczycki   +48 more
core   +2 more sources

Structural Characterization of the Enzymes Composing the Arginine Deiminase Pathway in Mycoplasma penetrans [PDF]

, 2012
The metabolism of arginine towards ATP synthesis has been considered a major source of energy for microorganisms such as Mycoplasma penetrans in anaerobic conditions.
Benach, Jordi, Gallego Alonso, Pablo, Planell Cerezo, Raquel, Pérez-Pons, Josep A., Querol Murillo, Enrique, Reverter i Cendrós, David   +5 more
core   +8 more sources

Fatal Hyperammonemic Brain Injury from Valproic Acid Exposure [PDF]

, 2012
Background: Hyperammonemia is known to cause neuronal injury, and can result from valproic acid exposure. Prompt reduction of elevated ammonia levels may prevent permanent neurological injury.
Bega, Danny, Boland, Torrey Ann, Chou, Sherry Hsiang-Yi, Murray, Michael, Vaitkevicius, Henrikas   +4 more
core   +2 more sources

Minimal ureagenesis is necessary for survival in the murine model of hyperargininemia treated by AAV-based gene therapy. [PDF]

, 2014
Hyperammonemia is less severe in arginase 1 deficiency compared with other urea cycle defects. Affected patients manifest hyperargininemia and infrequent episodes of hyperammonemia. Patients typically suffer from neurological impairment with cortical and
Cantero, G, Cantero-Nieto, G, Cederbaum, SD, Chan, E, Hu, C, Lipshutz, GS, Park, H, Tai, DS, Yudkoff, M   +8 more
core   +2 more sources

Association between the blood concentrations of ammonia and carnitine/amino acid of schizophrenic patients treated with valproic acid

BioPsychoSocial Medicine, 2017
Background Administration of valproic acid (VPA) is complicated with approximately 0.9% of patients developing hyperammonemia, but the pathogenesis of this adverse effect remains to be clarified.
Masazumi Ando, Hideaki Amayasu, Takahiro Itai, Hisahiro Yoshida   +3 more
doaj   +1 more source

Arginine Metabolism in Bacterial Pathogenesis and Cancer Therapy [PDF]

, 2016
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Botelho, MG, Lau, SKP, Lo, CLR, Teng, LL, Woo, PCY, Xiong, L   +5 more
core   +1 more source

N‐carbamoylglutamate‐responsive carbamoyl phosphate synthetase 1 (CPS1) deficiency: A patient with a novel CPS1 mutation and an experimental study on the mutation's effects

JIMD Reports, 2019
N‐carbamoyl‐l‐glutamate (NCG), the N‐acetyl‐l‐glutamate analogue used to treat N‐acetylglutamate synthase deficiency, has been proposed as potential therapy of carbamoyl phosphate synthetase 1 deficiency (CPS1D).
Sufin Yap, Nadine Gougeard, Anthony R. Hart, Belén Barcelona, Vicente Rubio   +4 more
doaj   +1 more source

Emergency Management of Intoxication-Type Inherited Metabolic Disorders. [PDF]

J Inherit Metab Dis
ABSTRACT In many intoxication‐type inherited metabolic disorders, the accumulation of the toxic chemical can cause acute life‐threatening emergencies. Sometimes this is the inevitable consequence of a severe metabolic defect, but it is often triggered by catabolism.
Tarr JD, Morris AAM.
europepmc   +2 more sources

Restoring Ureagenesis in Hepatocytes by CRISPR/Cas9-mediated Genomic Addition to Arginase-deficient Induced Pluripotent Stem Cells. [PDF]

, 2016
Urea cycle disorders are incurable enzymopathies that affect nitrogen metabolism and typically lead to hyperammonemia. Arginase deficiency results from a mutation in Arg1, the enzyme regulating the final step of ureagenesis and typically results in ...
Angarita, Stephanie Ak, Byrne, James A, Cederbaum, Stephen D, Chang, Katherine M, Gilmore, W Blake, Hermann, Kip, Lam, Alex K, Lee, Patrick C, Lipshutz, Gerald S, Pyle, April D, Schoenberg, Benjamen E, Tang, Jonathan K, Truong, Brian, Vega-Crespo, Agustin, Wininger, Austin E   +14 more
core   +3 more sources

White adipose tissue urea cycle activity is not affected by one-month treatment with a hyperlipidic diet in female rats. [PDF]

, 2016
Under high-energy diets, amino acid N is difficult to dispose of, as a consequence of the availability of alternative substrates. We found, recently, that WAT contains a complete functional urea cycle, we analyzed the possible overall changes in the WAT ...
Agnelli, Silvia, Alemany, Marià, 1946-, Arriarán, Sofía, Fernández López, José Antonio, Remesar Betlloch, Xavier   +4 more
core   +1 more source