Hepatic oxylipin profiles in mouse models of Wilson disease: New insights into early hepatic manifestations
We examined hepatic OXL profiles at early stages of WD in two mouse models, the toxic milk mouse from The Jackson Laboratory (tx-j) and the Atp7b knockout on a C57Bl/6 background (Atp7b-/-B6). Targeted lipidomic analysis performed by ultra-high-performance liquid chromatography-electrospray ionization-tandem mass spectrometry showed that in both tx-j and Atp7b-/-B6 mice, hepatic OXL profiles were altered with higher thromboxane and prostaglandins levels. The levels of oxidative stress marker, 9-HETE were increased more markedly in tx-j mice. However, both genotypes showed upregulated transcript levels of many genes related...
Source: Biochimica et Biophysica Acta - December 10, 2023 Category: Biochemistry Authors: Tagreed A Mazi Noreene M Shibata Gaurav V Sarode Valentina Medici Source Type: research
Hepatic oxylipin profiles in mouse models of Wilson disease: New insights into early hepatic manifestations
We examined hepatic OXL profiles at early stages of WD in two mouse models, the toxic milk mouse from The Jackson Laboratory (tx-j) and the Atp7b knockout on a C57Bl/6 background (Atp7b-/-B6). Targeted lipidomic analysis performed by ultra-high-performance liquid chromatography-electrospray ionization-tandem mass spectrometry showed that in both tx-j and Atp7b-/-B6 mice, hepatic OXL profiles were altered with higher thromboxane and prostaglandins levels. The levels of oxidative stress marker, 9-HETE were increased more markedly in tx-j mice. However, both genotypes showed upregulated transcript levels of many genes related...
Source: Mol Biol Cell - December 10, 2023 Category: Molecular Biology Authors: Tagreed A Mazi Noreene M Shibata Gaurav V Sarode Valentina Medici Source Type: research
Effects of tetrathiomolybdate on copper metabolism in healthy volunteers and in patients with Wilson disease
In Wilson Disease (WD), copper accumulates in the liver and brain causing disease. Bis-choline tetrathiomolybdate (TTM) is a potent copper chelator that may have a lower risk of inducing paradoxical neurological worsening than conventional therapy for neurologic WD. To better understand the mode of action of TTM, we investigated the effects of TTM on copper absorption and biliary excretion. (Source: Journal of Hepatology)
Source: Journal of Hepatology - December 9, 2023 Category: Gastroenterology Authors: Frederik Teicher Kirk, Ditte Emilie Munk, Eugene Scott Swenson, Adam M. Quicquaro, Mikkel Holm Vendelbo, Agnete Larsen, Michael L. Schilsky, Peter Ott, Thomas Damgaard Sandahl Tags: Research Article Source Type: research
Nano-Mediated Molecular Targeting in Diagnosis and Mitigation of Wilson Disease
AbstractWilson disease, a rare genetic disorder resulting from mutations in the ATP7B gene disrupts copper metabolism, leading to its harmful accumulation in hepatocytes, the brain, and other organs. It affects roughly 1 in 30,000 individuals, with 1 in 90 being gene carriers. Beyond gene mutations, the disease involves complex factors contributing to copper imbalance. Ongoing research seeks to unravel intricate molecular pathways, offering fresh insights into the disease ’s mechanisms. Simultaneously, there is a dedicated effort to develop effective therapeutic strategies. Nanotechnology-driven formulations are showing ...
Source: Molecular Neurobiology - December 8, 2023 Category: Neurology Source Type: research
The volume and structural covariance network of thalamic nuclei in patients with Wilson ’s disease: an investigation of the association with neurological impairment
ConclusionsThe findings suggest that the majority of thalamic nuclei in WDs exhibit significant volume reduction, and the atrophy of the right MDm is closely related to the severity of neurological symptoms. The intrinsic thalamic network in WDs demonstrated abnormal topological properties, indicating a close relationship with neurological impairment. (Source: Neurological Sciences)
Source: Neurological Sciences - December 5, 2023 Category: Neurology Source Type: research
Insights Into the Role of Copper in Neurodegenerative Diseases and the Therapeutic Potential of Natural Compounds
Curr Neuropharmacol. 2023 Nov 15. doi: 10.2174/1570159X22666231103085859. Online ahead of print.ABSTRACTNeurodegenerative diseases encompass a collection of neurological disorders originating from the progressive degeneration of neurons, resulting in the dysfunction of neurons. Unfortunately, effective therapeutic interventions for these diseases are presently lacking. Copper (Cu), a crucial trace element within the human body, assumes a pivotal role in various biological metabolic processes, including energy metabolism, antioxidant defense, and neurotransmission. These processes are vital for the sustenance, growth, and d...
Source: Current Neuropharmacology - December 1, 2023 Category: Drugs & Pharmacology Authors: Guangcheng Zhong Xinyue Wang Jiaqi Li Zhouyuan Xie Qiqing Wu Jiaxin Chen Yiyun Wang Ziying Chen Xinyue Cao Tianyao Li Jinman Liu Qi Wang Source Type: research
Insights Into the Role of Copper in Neurodegenerative Diseases and the Therapeutic Potential of Natural Compounds
Curr Neuropharmacol. 2023 Nov 15. doi: 10.2174/1570159X22666231103085859. Online ahead of print.ABSTRACTNeurodegenerative diseases encompass a collection of neurological disorders originating from the progressive degeneration of neurons, resulting in the dysfunction of neurons. Unfortunately, effective therapeutic interventions for these diseases are presently lacking. Copper (Cu), a crucial trace element within the human body, assumes a pivotal role in various biological metabolic processes, including energy metabolism, antioxidant defense, and neurotransmission. These processes are vital for the sustenance, growth, and d...
Source: Current Neuropharmacology - December 1, 2023 Category: Drugs & Pharmacology Authors: Guangcheng Zhong Xinyue Wang Jiaqi Li Zhouyuan Xie Qiqing Wu Jiaxin Chen Yiyun Wang Ziying Chen Xinyue Cao Tianyao Li Jinman Liu Qi Wang Source Type: research
Insights Into the Role of Copper in Neurodegenerative Diseases and the Therapeutic Potential of Natural Compounds
Curr Neuropharmacol. 2023 Nov 15. doi: 10.2174/1570159X22666231103085859. Online ahead of print.ABSTRACTNeurodegenerative diseases encompass a collection of neurological disorders originating from the progressive degeneration of neurons, resulting in the dysfunction of neurons. Unfortunately, effective therapeutic interventions for these diseases are presently lacking. Copper (Cu), a crucial trace element within the human body, assumes a pivotal role in various biological metabolic processes, including energy metabolism, antioxidant defense, and neurotransmission. These processes are vital for the sustenance, growth, and d...
Source: Current Neuropharmacology - December 1, 2023 Category: Drugs & Pharmacology Authors: Guangcheng Zhong Xinyue Wang Jiaqi Li Zhouyuan Xie Qiqing Wu Jiaxin Chen Yiyun Wang Ziying Chen Xinyue Cao Tianyao Li Jinman Liu Qi Wang Source Type: research
Insights Into the Role of Copper in Neurodegenerative Diseases and the Therapeutic Potential of Natural Compounds
Curr Neuropharmacol. 2023 Nov 15. doi: 10.2174/1570159X22666231103085859. Online ahead of print.ABSTRACTNeurodegenerative diseases encompass a collection of neurological disorders originating from the progressive degeneration of neurons, resulting in the dysfunction of neurons. Unfortunately, effective therapeutic interventions for these diseases are presently lacking. Copper (Cu), a crucial trace element within the human body, assumes a pivotal role in various biological metabolic processes, including energy metabolism, antioxidant defense, and neurotransmission. These processes are vital for the sustenance, growth, and d...
Source: Current Neuropharmacology - December 1, 2023 Category: Drugs & Pharmacology Authors: Guangcheng Zhong Xinyue Wang Jiaqi Li Zhouyuan Xie Qiqing Wu Jiaxin Chen Yiyun Wang Ziying Chen Xinyue Cao Tianyao Li Jinman Liu Qi Wang Source Type: research
Insights Into the Role of Copper in Neurodegenerative Diseases and the Therapeutic Potential of Natural Compounds
Curr Neuropharmacol. 2023 Nov 15. doi: 10.2174/1570159X22666231103085859. Online ahead of print.ABSTRACTNeurodegenerative diseases encompass a collection of neurological disorders originating from the progressive degeneration of neurons, resulting in the dysfunction of neurons. Unfortunately, effective therapeutic interventions for these diseases are presently lacking. Copper (Cu), a crucial trace element within the human body, assumes a pivotal role in various biological metabolic processes, including energy metabolism, antioxidant defense, and neurotransmission. These processes are vital for the sustenance, growth, and d...
Source: Current Neuropharmacology - December 1, 2023 Category: Drugs & Pharmacology Authors: Guangcheng Zhong Xinyue Wang Jiaqi Li Zhouyuan Xie Qiqing Wu Jiaxin Chen Yiyun Wang Ziying Chen Xinyue Cao Tianyao Li Jinman Liu Qi Wang Source Type: research
Insights Into the Role of Copper in Neurodegenerative Diseases and the Therapeutic Potential of Natural Compounds
Curr Neuropharmacol. 2023 Nov 15. doi: 10.2174/1570159X22666231103085859. Online ahead of print.ABSTRACTNeurodegenerative diseases encompass a collection of neurological disorders originating from the progressive degeneration of neurons, resulting in the dysfunction of neurons. Unfortunately, effective therapeutic interventions for these diseases are presently lacking. Copper (Cu), a crucial trace element within the human body, assumes a pivotal role in various biological metabolic processes, including energy metabolism, antioxidant defense, and neurotransmission. These processes are vital for the sustenance, growth, and d...
Source: Current Neuropharmacology - December 1, 2023 Category: Drugs & Pharmacology Authors: Guangcheng Zhong Xinyue Wang Jiaqi Li Zhouyuan Xie Qiqing Wu Jiaxin Chen Yiyun Wang Ziying Chen Xinyue Cao Tianyao Li Jinman Liu Qi Wang Source Type: research
Insights Into the Role of Copper in Neurodegenerative Diseases and the Therapeutic Potential of Natural Compounds
Curr Neuropharmacol. 2023 Nov 15. doi: 10.2174/1570159X22666231103085859. Online ahead of print.ABSTRACTNeurodegenerative diseases encompass a collection of neurological disorders originating from the progressive degeneration of neurons, resulting in the dysfunction of neurons. Unfortunately, effective therapeutic interventions for these diseases are presently lacking. Copper (Cu), a crucial trace element within the human body, assumes a pivotal role in various biological metabolic processes, including energy metabolism, antioxidant defense, and neurotransmission. These processes are vital for the sustenance, growth, and d...
Source: Current Neuropharmacology - December 1, 2023 Category: Drugs & Pharmacology Authors: Guangcheng Zhong Xinyue Wang Jiaqi Li Zhouyuan Xie Qiqing Wu Jiaxin Chen Yiyun Wang Ziying Chen Xinyue Cao Tianyao Li Jinman Liu Qi Wang Source Type: research
Insights Into the Role of Copper in Neurodegenerative Diseases and the Therapeutic Potential of Natural Compounds
Curr Neuropharmacol. 2023 Nov 15. doi: 10.2174/1570159X22666231103085859. Online ahead of print.ABSTRACTNeurodegenerative diseases encompass a collection of neurological disorders originating from the progressive degeneration of neurons, resulting in the dysfunction of neurons. Unfortunately, effective therapeutic interventions for these diseases are presently lacking. Copper (Cu), a crucial trace element within the human body, assumes a pivotal role in various biological metabolic processes, including energy metabolism, antioxidant defense, and neurotransmission. These processes are vital for the sustenance, growth, and d...
Source: Current Neuropharmacology - December 1, 2023 Category: Drugs & Pharmacology Authors: Guangcheng Zhong Xinyue Wang Jiaqi Li Zhouyuan Xie Qiqing Wu Jiaxin Chen Yiyun Wang Ziying Chen Xinyue Cao Tianyao Li Jinman Liu Qi Wang Source Type: research
EPH174 Trends in Hospitalization of Patients with Wilson's Disease in Germany
Wilson's disease (MW) is an autosomal recessive inherited disorder. Copper excretion from the liver via the bile duct is decreased due to gene mutations. Thus, copper accumulates in various organs, damaging them and leading to a variable symptom pattern. In recent years, new drugs have been approved for treatment. Here, we aim to investigate how frequently these therapies are used in inpatient settings and how hospitalization patterns have evolved since then. (Source: Value in Health)
Source: Value in Health - December 1, 2023 Category: International Medicine & Public Health Authors: S. Wahler, J. Oh, A. M üller, C. Koll, K.H. Weiss Source Type: research
EE398 Economic Burden of Wilson's Disease: A Systematic Review
This study summarizes the available evidence on its economic burden. (Source: Value in Health)
Source: Value in Health - December 1, 2023 Category: International Medicine & Public Health Authors: J. Garg, A. Mandal, I.S. Khurana, A. Ahuja Source Type: research
