Results 71 to 80 of about 1,630 (179)

Research progress in stem cell therapy for Wilson disease

Regenerative Therapy
Wilson disease (WD), also known as hepatolenticular degeneration, is an autosomal recessive disorder characterized by disorganized copper metabolism caused by mutations in the ATP7B gene.
Xianlang Xiong, Ce Gao, Xiangying Meng, Aihui Liu, Xin Gong, Yi Sun   +5 more
doaj   +1 more source

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

The production of pharmaceutical grade trientine dihydrochloride for the treatment of Wilson’s disease: a personal account

, 2016
This article describes the background to the production of trientine dihydrochloride for the treatment of Wilson's ...
Rupert Purchase (16039694)
core   +1 more source

Advance in the pathogenesis and treatment of Wilson disease

Translational Neurodegeneration, 2012
Wilson disease is an autosomal recessive disorder of copper metabolism. Diagnosis depends primarily on clinical features, biochemical parameters and the presence of the Kayser-Fleischer ring.
Dong Qin-Yun, Wu Zhi-Ying
doaj   +1 more source

Co-administration of trientine and flaxseed oil on oxidative stress, serum lipids and heart structure in diabetic rats [PDF]

, 2013
The administration of flaxseed oil or flaxseed oil plus trientine in diabetic rats reduced triglyceride, very low density lipoprotein, and total cholesterol.
Heidarian, E., Rezaei, A.
core   +1 more source

Trientine, A Copper-Chelating Agent, Induced Apoptosis in Murine Fibrosarcoma Cells in vivo and in vitro [PDF]

ArticleAnti-copper treatments have been investigated to determine whether they suppress angiogenesis and tumor development since Cu is widely accepted as being required for angiogenesis.
HAYASHI, Masanobu, 7530, 9420, CHIBA, Toshiaki, NISHIYA, Hide, ENDO, Daiji, 遠藤, 大二, KON, Yasuhiro, OKUI, Toyo, 7527, 7526, 4569, 7529, 7528   +13 more
core  

Trientine for hypertrophic cardiomyopathy: a phase 2 trial [PDF]

Background and Aims Pathophysiological features of hypertrophic cardiomyopathy include left ventricular hypertrophy, myocardial fibrosis, and myocardial energy deficiency.
Thomas Green, Dana Dawson, Anna B Reid, Marc R Dweck, Anna Rosala-Hallas, Matthias Schmitt, Masliza Mahmod, Olatz Baroja, Emma Bedson, Beatriz Duran, Carly Vaughan, Dannii Clayton, Josephine Naish, Betty Raman, Colin Berry, Zakariye Ashkir, Sanjay Prasad, Saidi Mohiddin, Robert Cooper, James C Moon, Sven Plein, John P Farrant, Ladislav Valkovič, Chris F Harrington, Christopher A Miller, Scot Garg, Nicola Greenlaw, Susanna Dodd, Mohammed Akhtar, Catherine Spowart, Anvesha Singh, Catriona Graham, Clifford J Garratt, Stefan Neubauer, Moon, James C, Cooper, R, Graham, Catriona, Neubauer, Stefan, Moon, James C., Baroja, Olatz, Garg, Scot, Spowart, Catherine, Graham, C, Singh, A, Farrant, John P., Spowart, C, Dawson, Dana, Mohiddin, Saidi, Naish, J, Plein, S, Raman, B, Prasad, Sanjay, Clayton, Dannii, Akhtar, Mohammed, Raman, Betty, Schmitt, Matthias, Schmitt, M, Ashkir, Z, Dweck, Marc R, Vaughan, C, Mahmod, Masliza, Dodd, S, Moon, JC, Bedson, Emma, Valkovic, L, Dawson, D, Berry, Colin, Ashkir, Zakariye, Berry, C, Neubauer, S, Garratt, Clifford J, Garratt, Clifford J., Miller, Christopher A, Farrant, John P, Harrington, Chris F, Baroja, O, Dweck, MR, Garg, S, Green, T, Singh, Anvesha, Vaughan, Carly, Harrington, CF, Rosala-Hallas, A, Clayton, D, Dweck, Marc R., Garratt, CJ, Greenlaw, N, Reid, Anna B, Naish, Josephine, Prasad, S, Mohiddin, S, Green, Thomas, Plein, Sven, Harrington, Chris F., Dodd, Susanna, Duran, Beatriz, Miller, Christopher A., Valkovic, Ladislav, Miller, CA, Greenlaw, Nicola, Bedson, E, Duran, B, Farrant, JP, Cooper, Robert, Mahmod, M, Akhtar, M, Rosala-Hallas, Anna, Reid, AB   +107 more
core   +1 more source

The Metal Chelators, Trientine and Citrate, Inhibit the Development of Cardiac Pathology in the Zucker Diabetic Rat [PDF]

, 2009
Purpose. The objective of this study was to determine the efficacy of dietary supplementation with the metal chelators, trientine or citric acid, in preventing the development of cardiomyopathy in the Zucker diabetic rat. Hypothesis. We hypothesized that
Murray, David B., Baynes, John W
core   +1 more source

A copper(II)-selective chelator ameliorates left-ventricular hypertrophy in type 2 diabetic patients: A randomised placebo-controlled study [PDF]

, 2009
Aims/hypothesis: Cu(II)-selective chelation with trientine ameliorates cardiovascular and renal disease in a model of diabetes in rats. Here, we tested the hypothesis that Cu(II)-selective chelation might improve left ventricular hypertrophy (LVH) in ...
Young, A. A., Dissanayake, A. M., Poppitt, S. D., Gamble, G. D., Frampton, C. M., Baker, J. R., Doughty, R. N., Cooper, Garth, Cowan, B. R., Phillips, A. R J, Occleshaw, C. J., Brunton, D. H.   +11 more
core   +1 more source

Treatment with a copper-selective chelator causes substantive improvement in cardiac function of diabetic rats with left-ventricular impairment [PDF]

, 2013
Background: Defective copper regulation is implicated as a causative mechanism of organ damage in diabetes. Treatment with trientine, a divalent-copper-selective chelator, improves arterial and renal structure/function in diabetes, wherein it also ...
Pickup, S, Cooper, Garth J S, Li, M, Cooper, GJ, Phillips, Anthony R J, Choong, SY, Pickup, Stephen, Brett R Cowan, Garth JS Cooper, Young, AA, Lu, Jun, Xu, H, Anthony RJ Phillips, Jun Lu, Choong, Soon Y, Pontré, Beau, Cowan, BR, Beau Pontré, Pontré, B, Gamble, GD, Mingming Li, Lu, J, Xu, Hong, Jun Lu, Li, Mingming, Soon Y Choong, Stephen Pickup, Hong Xu, Gregory D Gamble, Cowan, Brett R., Young, Alistair A., Alistair A Young, Gamble, Gregory D., Choong, Soon Y., Phillips, AR, Young, Alistair A, Gamble, Gregory D, Phillips, Anthony RJ, Cowan, Brett R, Cooper, Garth JS   +39 more
core   +1 more source