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Showing 1 to 6 of 6 for “"hyperammonemia"”.

  1. DNA analysis of Ornithine Transcarbamylase (OTC) deficiency in South African patients

    … later in infancy, childhood and adulthood with hyperammonemia and episodic mental status changes. The fifth defect, arginase deficiency presents as progressive spastic quadriplegia and mental retardation but with milder elevation of blood ammonia levels. The molecular genetics of these disorders …

    cape-town Repository record for DNA analysis of Ornithine Transcarbamylase (OTC) deficiency in South African patients (opens in a new tab)

  2. Developing disease-state liver models to determine the effects on brain function in vitro

    … neurotransmitter production when exposed to the hyperammonemia conditions present during hepatic encephalopathy.

    uiuc Repository record for Developing disease-state liver models to determine the effects on brain function in vitro (opens in a new tab)

  3. Thermodynamics of Ligand Binding to Glutamate Dehydrogenase

    … hyperinsulinism, specifically, hyperinsulinism/hyperammonemia syndrome (HHS). GDH catalyzes the reversible deamination of glutamate to 2-oxoglutarate. Mutations in GDH can lead to GDH over activity, causing increased ATP production via the Krebs Cycle and excess insulin release. In addition, GDH …

    utmb Repository record for Thermodynamics of Ligand Binding to Glutamate Dehydrogenase (opens in a new tab)

  4. The Role of Gut Microbiota Urease in the Host With Liver Disease

    … the normal processing of ammonia, leading to hyperammonemia and hepatic encephalopathy (HE). Although circulating ammonia levels are correlated with damage to the central nervous system, the pathogenesis of HE is complex and not fully elucidated, hindering progress in treatment. Current …

    penn Repository record for The Role of Gut Microbiota Urease in the Host With Liver Disease (opens in a new tab)

  5. Interrogation of the human glutamate dehydrogenase antenna to elucidate its role in allosteric regulation and disease

    … regulation is made evident by hyperinsulinism/hyperammonemia syndrome (HI/HA), where mutations cause GDH hyperactivity. Since only allosterically regulated forms of the enzyme contain an approximately 50-residue antenna domain, it suggests that the antenna is critical for allosteric regulation …

    utmb Repository record for Interrogation of the human glutamate dehydrogenase antenna to elucidate its role in allosteric regulation and disease (opens in a new tab)

  6. Kontinuierliches Monitoring von Glukose, Laktat und Ammonium mit 'Bioanalytischen Mikrosystemen'

    Ein kontinuierliches und simultanes Monitoring von Glukose und Laktat im subkutanen Gewebe konnte durch die Kombination der “Mikrodialyse“ als Probennahme-System mit einem “Bioanalytischen Mikrosystem“ mit integrierten Biosensoren als Analysemethode ermöglicht werden. Es konnte gezeigt werden, dass …

    freiburg-diss Repository record for Kontinuierliches Monitoring von Glukose, Laktat und Ammonium mit 'Bioanalytischen Mikrosystemen' (opens in a new tab)