Results 61 to 70 of about 1,630 (179)

The development of trientine dihydrochloride, 1977-85

, 1986
Key dates in the development of a laboratory and pilot plant route for pharmaceutical grade trientine dihydrochloride are ...
Roger Humphreys (16281365), Rupert Purchase (16039694)   +1 more
core   +1 more source

Insights into the management of Wilson’s disease

Therapeutic Advances in Gastroenterology, 2017
Wilson’s disease is a rare, inherited autosomal recessive disease of copper metabolism, in which the causative gene, ATP7B , results in absent or reduced function of the ATP7B transporter important for biliary excretion of copper and incorporation of ...
Mohmadshakil Kathawala, Gideon M. Hirschfield   +1 more
doaj   +1 more source

From Radiocopper to Cold Copper: Mechanistic Modeling and Simulation to Define Clinical Response Criteria and Biomarkers for VTX‐801 in Wilson Disease

CPT: Pharmacometrics &Systems Pharmacology, Volume 15, Issue 1, January 2026.
ABSTRACT We developed a comprehensive, mechanistic model of human copper metabolism to support biomarker qualification for VTX‐801, an adeno‐associated vector‐based gene therapy which is being developed to restore the mutated ATP7B copper transporter gene in Wilson disease (WD). The model integrates physiological copper kinetics with pathophysiological
Andreas Lindauer, Bernard Benichou, Gloria González Aseguinolaza, Jean‐Philippe Combal   +3 more
wiley   +1 more source

Methanobactin rapidly facilitates biliary copper excretion in a Wilson disease rat model visualised by 64Cu PET/MRI

British Journal of Pharmacology, Volume 183, Issue 2, Page 268-279, January 2026.
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 model, compared to penicillamine or saline, by 64Cu positron emission tomography/magnetic resonance imaging ...
Emilie Munk Lynderup, Mikkel Holm Vendelbo, Frederik Teicher Kirk, Karina Højrup Vase, Aage Kristian Olsen Alstrup, Tamara Rieder, Alan A. DiSpirito, Jeremy D. Semrau, Tea Lund Laursen, Peter Ott, Hans Zischka, Thomas Damgaard Sandahl   +11 more
wiley   +1 more source

Cuproptosis at the Crossroads of Tumor Microenvironment Inflammation and Metabolic Rewiring Mechanisms and Therapeutic Opportunities

Mediators of Inflammation, Volume 2026, Issue 1, 2026.
Metabolic reorganization and chronic inflammation are dynamic and diverse events in the tumor microenvironment (TME) that cause tumor propagation, spreading, and resistance to treatment. However, copper‐induced cell death (cuproptosis), which is a newly discovered form of regulated cell death (RCD) induced by high intracellular copper ions and is a ...
Yan Wang, Yunfei Zhu, Yanxin Wang, Yutong Liu, Pengfei Wang, Mingkun Yu, Hengrui Liu   +6 more
wiley   +1 more source

Plasma Glial Fibrillary Acidic Protein Correlates With Brain Metal Burden in Wilson's Disease

Annals of Clinical and Translational Neurology, Volume 12, Issue 12, Page 2433-2445, December 2025.
ABSTRACT Objective Neuroinflammation driven by extracellular copper contributes to neuronal damage in Wilson's disease (WD). This study investigated the relationship between brain metal burden and peripheral neuroinflammation markers in WD. Methods We conducted a cross‐sectional study involving 89 participants, including patients with WD (n = 63 ...
Sung‐Pin Fan, Ya‐Fang Chen, Cheng‐Hsuan Li, Yih‐Chih Kuo, Ni‐Chung Lee, Yin‐Hsiu Chien, Wuh‐Liang Hwu, Tai‐Chung Tseng, Tung‐Hung Su, Chien‐Ting Hsu, Huey‐Ling Chen, Chin‐Hsien Lin, Yen‐Hsuan Ni   +12 more
wiley   +1 more source

Copper(I)-binding properties of de-coppering drugs for the treatment of Wilson disease. α-Lipoic acid as a potential anti-copper agent

Scientific Reports, 2018
Wilson disease is an autosomal recessive genetic disorder caused by loss-of-function mutations in the P-type copper ATPase, ATP7B, which leads to toxic accumulation of copper mainly in the liver and brain. Wilson disease is treatable, primarily by copper-
Julia Smirnova, Ekaterina Kabin, Ivar Järving, Olga Bragina, Vello Tõugu, Thomas Plitz, Peep Palumaa   +6 more
doaj   +1 more source

COL6A2: A Key Survival‐Related Gene and Restricting Antitumor Immunity in Glioblastoma

Cancer Science, Volume 116, Issue 12, Page 3487-3502, December 2025.
Targeting COL6A2 enhances the activation of dendritic cells and anti‐tumor immunity in glioblastoma multiforme (GBM). COL6A2 may act as a key mediator of immune escape in GBM and a potential target for combinatorial immunotherapeutic strategies. ABSTRACT Glioblastoma (GBM) is an aggressive malignant brain tumor, characterized by a poor prognosis and a ...
Zhenkun Yang, Jiao Meng, Daxing Xu, Jie Li, Fan Kong, Ying Yin, Bo Zhang, Yunhui Pan, Jiantong Jiao, Xinyi Jiang, Zhening Pu   +10 more
wiley   +1 more source

Comprehensive Pharmacological Management of Wilson’s Disease: Mechanisms, Clinical Strategies, and Emerging Therapeutic Innovations

Sci
Wilson’s disease is a rare autosomal recessive disorder of copper metabolism characterized by excessive copper accumulation in the liver, brain, and other tissues.
Ralf Weiskirchen
doaj   +1 more source

Clinical Features and Therapeutic Response in Taiwanese Children With Wilson's Disease: 12 Years of Experience in a Single Center

Pediatrics and Neonatology, 2010
Wilson's disease (WD) is an autosomal recessive defect of cellular copper export. Early diagnosis in children is difficult due to its obscure clinical presentations.
Li-Ching Wang, Jiaan-Der Wang, Chi-Ren Tsai, Shao-Bin Cheng, Chieh-Chung Lin   +4 more
doaj   +1 more source