Results 71 to 80 of about 2,712 (224)

Uncovering the Critical Role of Cuproptosis in Wilson Disease: Insights Into Potential Therapeutic Targets

Journal of Cellular and Molecular Medicine, Volume 29, Issue 21, November 2025.
ABSTRACT Wilson disease (WD) is an inherited disorder caused by ATP7B mutations, resulting in toxic copper accumulation primarily in the liver and brain. While copper‐induced hepatotoxicity is a hallmark of WD, the mechanisms linking copper overload to liver injury remain unclear.
Shan Tang, Feng Ren, Wei Hou, Zihao Fan, Yinkang Mo, Xianru Zhu, Yaling Cao, Ling Xu, Sujun Zheng   +8 more
wiley   +1 more source

Targeting Intratumoral Copper Inhibits Tumor Progression via p62‐Mediated EZH2 Degradation and Potentiates Anti‐PD‐1 Immunotherapy in Oral Squamous Cell Carcinoma

Advanced Science, Volume 12, Issue 40, October 27, 2025.
The authors find that by targeting intratumoral copper, they can enhance p62‐mediated ubiquitination of EZH2 at the Ub‐K63 site by suppressing copper binding to SMURF2, an E3 ligase of EZH2, leading to its autophagic degradation. This mechanism suppressed OSCC progression and potentiated anti‐PD‐1 immunotherapy, highlighting a potential new therapeutic
Xiaohu Lin, Wanling Chen, Bo Li, Zhang Zhao, Zhonglin Yu, Xu‐Yun Zhao, Xuan Zhou, Zhien Feng, Chengzhong Lin, Wei Cao   +9 more
wiley   +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

Chelators in Iron and Copper Toxicity [PDF]

Purpose of Review Chelation therapy is used for diseases causing an imbalance of iron levels (for example haemochromatosis and thalassaemia) or copper levels (for example Menkes’ and Wilson’s diseases).
A Hartwig, A Kattamis, A Miyashiro, AC Dlouhy, AV Hoffbrand, B Gupta, BE Holbein, BE Kim, BM Olabinri, C Thephinlap, CG Roussaeux, CL Alden, CW Ritchie, DJ Kosman, DM Seely, DS Kalinowski, DW Cox, ED Rosenstein, G Lewis, GE Cartwright, GJ Brewer, HV Aposian, IS MacPherson, J Aaseth, J Aaseth, JH Menkes, JM Walshe, JM Walshe, K Aayush, K Brzóska, K Grasedyck, K Jomova, KA Winship, L Alan, L Lannfelt, M Nairz, MD Cappellini, MG Thompson, ML Schilsky, P Das, P Delangle, PJ Crouch, PJ Giardina, RL Anderson, RM Robins-Browne, RR Crichton, S Srichairatanakool, S Waldvogel-Abramowskia, SJS Flora, SK Das, SR Bareggi, T Umemura, TA Rouault, TD Rae, TP Kennedy, U Merle, V Laufberger, V Oliveri, Y Hiasa   +58 more
core   +1 more source

Comparative effectiveness of common therapies for Wilson disease: A systematic review and meta-analysis of controlled studies [PDF]

, 2019
BACKGROUND & AIMS: Wilson disease (WD) is a rare disorder of copper metabolism. The objective of this systematic review is to determine the comparative effectiveness and safety of common treatments of WD.
Aggarwal A, Cacic M, Chen JL, Chen JL, Chen ZR, Czlonkowska A, Hu Z, Jablonska‐Kaszewska I, Lee DY, Li N, Nagy J, Strickland GT, Szleper M, Vieira Barbosa J, Xie XW, Yuzbasiyan‐Gurkan V   +15 more
core   +1 more source

Predictive Analysis for First Submission of Generic Drug Application for Orphan Drug Products Using Random Survival Forest

Clinical and Translational Science, Volume 18, Issue 10, October 2025.
ABSTRACT Rare diseases affect a small population of patients, resulting in low incentives for developing orphan drug products (ODPs). The United States Congress passed the Orphan Drug Act of 1983 to incentivize pharmaceutical manufacturers to develop drugs to treat rare diseases.
Robert Hopefl, Jing Wang, Abhinav Ram Mohan, Wei‐Jhe Sun, Myong‐Jin Kim, Meng Hu, Lanyan Fang   +6 more
wiley   +1 more source

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

Mediators Inflamm
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 ...
Wang Y, Zhu Y, Wang Y, Liu Y, Wang P, Yu M.   +5 more
europepmc   +2 more sources

Update on Genetic Chorea

European Journal of Neurology, Volume 32, Issue 10, October 2025.
This review provides an updated clinical and genetic framework for the differential diagnosis of hereditary chorea. It guides neurologists through the interpretation of phenomenology, ancillary tests, and appropriate genetic techniques to achieve an accurate and timely diagnosis.
Jesús Pérez‐Pérez, Gonzalo Olmedo‐Saura, Saül Martínez‐Horta, Sara Bernal, Javier Pagonabarraga, Jaime Kulisevsky   +5 more
wiley   +1 more source

The Regulation of Trace Metal Elements in Cancer Ferroptosis

Advanced Biology, Volume 9, Issue 8, August 2025.
The induction of ferroptosis inhibits tumor growth, enhances anticancer efficacy, and overcomes drug resistance. Recent evidence shows nonferrous metal elements play a role in ferroptosis. This review focuses on how trace metals regulate ferroptosis processes like iron accumulation, lipid peroxidation, and antioxidant defense.
Xiaoyan Wang, Yuanyuan Xue, Lei Chang, Xuena Zhu, Wenjun Liu, Tingbo Liang   +5 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