Peter Ott
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View article: Methanobactin rapidly facilitates biliary copper excretion in a Wilson disease rat model visualised by <sup>64</sup>Cu PET/MRI
Methanobactin rapidly facilitates biliary copper excretion in a Wilson disease rat model visualised by <sup>64</sup>Cu PET/MRI Open
Background and Purpose Methanobactins are peptides with high copper affinity and potential to treat Wilson disease. We examined how two methanobactins (ARBM101 and MB‐OB3b) affected copper handling in the LPP Atp7b −/− Wilson disease rat m…
View article: Relative Exchangeable Copper, Exchangeable Copper and Total Copper in the Diagnosis of Wilson Disease
Relative Exchangeable Copper, Exchangeable Copper and Total Copper in the Diagnosis of Wilson Disease Open
Background and Aims Diagnosing Wilson disease (WD) remains challenging. The exchangeable copper (CuEXC) methodology measures the non‐ceruloplasmin‐bound copper fraction in serum. Relative exchangeable copper (REC), the ratio of CuEXC to to…
View article: Down the road towards hepatic encephalopathy. The elusive ammonia– what determines the arterial concentration?
Down the road towards hepatic encephalopathy. The elusive ammonia– what determines the arterial concentration? Open
View article: Down the road towards hepatic encephalopathy. Urea synthesis - the liver workhorse of nitrogen metabolism
Down the road towards hepatic encephalopathy. Urea synthesis - the liver workhorse of nitrogen metabolism Open
View article: Blood ammonia concentration measurement – effects of sampling site and cirrhosis during induced hyperammonaemia
Blood ammonia concentration measurement – effects of sampling site and cirrhosis during induced hyperammonaemia Open
Background Ammonia is implicated in hepatic encephalopathy (HE) and prognostic in cirrhosis. Venous ammonia concentration, yielding similar correlation with HE grades as arterial, has become the preferred practise but comparative data are …
View article: Hepato‐splanchnic fluxes during exercise in patients with cirrhosis—a pilot study
Hepato‐splanchnic fluxes during exercise in patients with cirrhosis—a pilot study Open
In cirrhotic patients, compromised hepatocyte function combined with disturbed hepatic blood flow could affect hepato‐splanchnic substrate and metabolite fluxes and exacerbate fatigue during exercise. Eight cirrhotic patients performed inc…
View article: Non-ceruloplasmin copper and urinary copper in clinically stable Wilson disease: Alignment with recommended targets
Non-ceruloplasmin copper and urinary copper in clinically stable Wilson disease: Alignment with recommended targets Open
View article: Blood ammonia concentration measurement – effects of sampling site and cirrhosis during induced hyperammonaemia
Blood ammonia concentration measurement – effects of sampling site and cirrhosis during induced hyperammonaemia Open
Background: Ammonia is implicated in hepatic encephalopathy (HE) and prognostic in cirrhosis. Venous ammonia concentration, yielding similar correlation with HE grades as arterial, has become the preferred practise but comparative data are…
View article: Diverse roles of the metal binding domains and transport mechanism of copper transporting P-type ATPases
Diverse roles of the metal binding domains and transport mechanism of copper transporting P-type ATPases Open
View article: Effects of trientine and penicillamine on intestinal copper uptake: A mechanistic 64Cu PET/CT study in healthy humans
Effects of trientine and penicillamine on intestinal copper uptake: A mechanistic 64Cu PET/CT study in healthy humans Open
Background and Aims: Trientine (TRI) and D-penicillamine (PEN) are used to treat copper overload in Wilson disease. Their main mode of action is thought to be through the facilitation of urinary copper excretion. In a recent study, TRI was…
View article: Effects of tetrathiomolybdate on copper metabolism in healthy volunteers and in patients with Wilson disease
Effects of tetrathiomolybdate on copper metabolism in healthy volunteers and in patients with Wilson disease Open
View article: Distribution of non-ceruloplasmin-bound copper after i.v. 64Cu injection studied with PET/CT in patients with Wilson disease
Distribution of non-ceruloplasmin-bound copper after i.v. 64Cu injection studied with PET/CT in patients with Wilson disease Open
View article: Positron Emission Tomography Using 64-Copper as a Tracer for the Study of Copper-Related Disorders
Positron Emission Tomography Using 64-Copper as a Tracer for the Study of Copper-Related Disorders Open
Copper is an essential trace element, functioning in catalysis and signaling in biological systems. Radiolabeled copper has been used for decades in studying basic human and animal copper metabolism and copper-related disorders, such as Wi…
View article: Clearance and production of ammonia quantified in humans by constant ammonia infusion – the effects of cirrhosis and ammonia-targeting treatments
Clearance and production of ammonia quantified in humans by constant ammonia infusion – the effects of cirrhosis and ammonia-targeting treatments Open
ClinicalTrials.gov (1-16-02-297-20).
View article: The association between soluble CD163, disease severity, and ursodiol treatment in patients with primary biliary cholangitis
The association between soluble CD163, disease severity, and ursodiol treatment in patients with primary biliary cholangitis Open
Introduction: The macrophage activation marker soluble (s)CD163 is associated with disease severity and prognosis in patients with primary biliary cholangitis (PBC). Ursodeoxycholic acid (UDCA) treatment attenuates fibrosis progression in …
View article: Trientine tetrahydrochloride versus penicillamine for maintenance therapy in Wilson disease (CHELATE): a randomised, open-label, non-inferiority, phase 3 trial
Trientine tetrahydrochloride versus penicillamine for maintenance therapy in Wilson disease (CHELATE): a randomised, open-label, non-inferiority, phase 3 trial Open
View article: Case report: Huppke–Brendel syndrome in an adult, mistaken for and treated as Wilson disease for 25 years
Case report: Huppke–Brendel syndrome in an adult, mistaken for and treated as Wilson disease for 25 years Open
Background Huppke–Brendel (HB) syndrome is an autosomal recessive disease caused by variants in the SLC33A1 gene. Since 2012, less than ten patients have been reported, none survived year six. With neurologic involvement and ceruloplasmin …
View article: Effect of oral zinc regimens on human hepatic copper content: a randomized intervention study
Effect of oral zinc regimens on human hepatic copper content: a randomized intervention study Open
Zinc inhibits intestinal copper uptake, an effect utilized for treating Wilson’s disease (WD). We used copper-64 ( 64 Cu) PET/CT to examine how much four weeks of treatment with different zinc regimens reduced the hepatic 64 Cu content aft…
View article: Celebration of Professor Bernhard Weigand on his 60th birthday
Celebration of Professor Bernhard Weigand on his 60th birthday Open
View article: Accuracy improvement of the transient heater foil technique for heat transfer tests: preliminary results
Accuracy improvement of the transient heater foil technique for heat transfer tests: preliminary results Open
Experimental heat transfer measurements are used in a wide range of fields, for example to validate new cooling concepts in turbomachinery, to assess the performances of heat exchangers, and to provide data for numerical simulations. A par…
View article: The pathophysiology of Wilson’s disease visualized: A human 64Cu PET study
The pathophysiology of Wilson’s disease visualized: A human 64Cu PET study Open
Background and Aims Wilson’s disease (WD) is a genetic disease with systemic accumulation of copper that leads to symptoms from the liver and brain. However, the underlying defects in copper transport kinetics are only partly understood. W…
View article: Transient liquid crystal thermography using a time varying surface heat flux
Transient liquid crystal thermography using a time varying surface heat flux Open
Heat transfer measurements are required in a wide range of fields, for example to validate new cooling concepts in turbomachinery, to assess the performances of heat exchangers, and to provide data for numerical simulations. Thereby transi…
View article: Designing Clinical Trials in Wilson’s Disease
Designing Clinical Trials in Wilson’s Disease Open
Background and Aims Wilson’s disease (WD) is an autosomal‐recessive disorder caused by ATP7B gene mutations leading to pathological accumulation of copper in the liver and brain. Adoption of initial treatments for WD was based on empirical…
View article: Glutamate Dehydrogenase Is Important for Ammonia Fixation and Amino Acid Homeostasis in Brain During Hyperammonemia
Glutamate Dehydrogenase Is Important for Ammonia Fixation and Amino Acid Homeostasis in Brain During Hyperammonemia Open
Impaired liver function may lead to hyperammonemia and risk for hepatic encephalopathy. In brain, detoxification of ammonia is mediated mainly by glutamine synthetase (GS) in astrocytes. This requires a continuous de novo synthesis of glut…
View article: Heat Transfer Enhancement in a Double Sequential Impingement Channel
Heat Transfer Enhancement in a Double Sequential Impingement Channel Open
Sequential impingement channels can reduce the adverse effect of crossflow in narrow impingement channels, as well as increase the cooling efficiency. In this work, sequential impingement channels are experimentally investigated using the …
View article: Cognitive Impairment In Stable Wilson Disease Across Phenotype.
Cognitive Impairment In Stable Wilson Disease Across Phenotype. Open
Background: In Wilson disease (WD), mutations in the gene encoding the ATP7B copper transport protein causes accumulation of copper especially in liver and brain. WD typically presents with hepatic and/or neuropsychiatric symptoms. Impaire…
View article: Hepatic bile acid transport increases in the postprandial state: A functional 11C-CSar PET/CT study in healthy humans
Hepatic bile acid transport increases in the postprandial state: A functional 11C-CSar PET/CT study in healthy humans Open
View article: Memory Traces Formed in Utero—Newborns’ Autonomic and Neuronal Responses to Prenatal Stimuli and the Maternal Voice
Memory Traces Formed in Utero—Newborns’ Autonomic and Neuronal Responses to Prenatal Stimuli and the Maternal Voice Open
In our pilot study, we exposed third-trimester fetuses, from week 34 of gestation onwards, twice daily to a maternal spoken nursery rhyme. Two and five weeks after birth, 34 newborns, who were either familiarized with rhyme stimulation in …
View article: Recovery of moderate COVID-19 disease in a liver transplant recipient on continued immunosuppression
Recovery of moderate COVID-19 disease in a liver transplant recipient on continued immunosuppression Open
The authors have requested that this preprint be withdrawn due to author disagreement.
View article: A placebo-controlled randomised trial of budesonide for PBC following an insufficient response to UDCA
A placebo-controlled randomised trial of budesonide for PBC following an insufficient response to UDCA Open