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  • Review
  • Open Access
18 Citations
11,751 Views
15 Pages

D-Penicillamine: The State of the Art in Humans and in Dogs from a Pharmacological and Regulatory Perspective

  • Michela Pugliese,
  • Vito Biondi,
  • Enrico Gugliandolo,
  • Patrizia Licata,
  • Alessio Filippo Peritore,
  • Rosalia Crupi and
  • Annamaria Passantino

Chelant agents are the mainstay of treatment in copper-associated hepatitis in humans, where D-penicillamine is the chelant agent of first choice. In veterinary medicine, the use of D-penicillamine has increased with the recent recognition of copper-...

  • Case Report
  • Open Access
1 Citations
2,960 Views
10 Pages

1 September 2025

Background and Clinical Significance: Cystinuria is the most common genetic cause of pediatric nephrolithiasis, characterized by impaired renal cystine reabsorption and resulting in increased urinary cystine excretion. Due to the poor solubility of c...

  • Article
  • Open Access
10 Citations
2,451 Views
14 Pages

31 January 2024

D-penicillamine (D-PA) is a sulfur-containing drug that has been used for various health conditions. However, like any medication, overdosing on D-PA can have adverse effects and may require additional treatment. Therefore, developing simple and sens...

  • Article
  • Open Access
22 Citations
5,517 Views
21 Pages

D-Penicillamine Reveals the Amelioration of Seizure-Induced Neuronal Injury via Inhibiting Aqp11-Dependent Ferroptosis

  • Nan Yang,
  • Kai Zhang,
  • Qi-Wen Guan,
  • Zhao-Jun Wang,
  • Kang-Ni Chen and
  • Xiao-Yuan Mao

19 August 2022

Repetitive seizures, a common phenomenon in diverse neurologic conditions such as epilepsy, can undoubtedly cause neuronal injury and our prior work reveals that ferroptosis is a contributing factor of neuronal damage post seizure. However, there is...

  • Article
  • Open Access
4 Citations
2,873 Views
16 Pages

22 January 2023

D-penicillamine (PA) is a sulfur group-containing drug prescribed for various health issues, but overdoses have adverse effects. Therefore, regular, selective, and sensitive sensing is essential to reduce the need for further treatment. In this study...

  • Article
  • Open Access
1 Citations
830 Views
18 Pages

28 December 2025

Polyphenols like dihydroquercetin, rutin, and rifampicin show promise for Alzheimer’s disease (AD) therapy due to their ability to inhibit amyloid-β (Aβ) aggregation and reduce reactive oxygen species (ROS), garnering significant rece...

  • Case Report
  • Open Access
10 Citations
3,569 Views
11 Pages

10 August 2023

Wilson’s disease (WD) is a genetic disorder with copper accumulation in various tissues leading to related clinical symptoms (mainly hepatic and neuropsychiatric) which can be in 85% of patients successfully treated with anti-copper agents. How...

  • Article
  • Open Access
25 Citations
6,061 Views
18 Pages

Analyzing the Therapeutic Efficacy of Bis-Choline-Tetrathiomolybdate in the Atp7b−/− Copper Overload Mouse Model

  • Philipp Kim,
  • Chengcheng Christine Zhang,
  • Sven Thoröe-Boveleth,
  • Eva Miriam Buhl,
  • Sabine Weiskirchen,
  • Wolfgang Stremmel,
  • Uta Merle and
  • Ralf Weiskirchen

Bis-choline-tetrathiomolybdate, introduced as WTX101 (now known as ALXN1840), is a first-in-class copper-protein-binding agent for oral therapy of Wilson’s disease. In contrast to other decoppering agents such as trientine or D-penicillamine it...

  • Article
  • Open Access

Morbus Wilson: eine klinische und molekularbiologische Kasuistik

  • Christoph Noppen,
  • J. Wyrsch,
  • E. H. Viollier,
  • K. Hess and
  • C. Schaefer

Wilson’s disease is a rare autosomal recessively inherited disorder of copper metabolism resulting in accumulation of copper in many organs. The Kayser-Fleischer corneal ring represents a typical symptom in Wilson patients. ATP7B, the gene product of...

  • Article
  • Open Access
7 Citations
4,028 Views
18 Pages

30 March 2022

Assessments of sperm performance are valuable tools for the analysis of sperm fertilizing potential and to understand determinants of male fertility. Hamster species constitute important animal models because they produce sperm cells in high quantiti...

  • Article
  • Open Access
9 Citations
3,570 Views
14 Pages

Effect of Copper Chelators via the TGF-β Signaling Pathway on Glioblastoma Cell Invasion

  • Heabin Kim,
  • Seonmi Jo,
  • In-Gyu Kim,
  • Rae-Kwon Kim,
  • Yeon-Jee Kahm,
  • Seung-Hyun Jung and
  • Jei Ha Lee

13 December 2022

Glioblastoma multiforme (GBM) is a fast-growing and aggressive type of brain cancer. Unlike normal brain cells, GBM cells exhibit epithelial–mesenchymal transition (EMT), which is a crucial biological process in embryonic development and cell m...

  • Article
  • Open Access
14 Citations
6,605 Views
13 Pages

Synthetic nitric oxide (NO)-donating materials have been shown to have many beneficial effects when incorporated into biomedical materials. When released in the correct dosage, NO has been shown to increase the biocompatibility of blood and tissue co...

  • Article
  • Open Access
15 Citations
4,653 Views
30 Pages

Copper Modulates Adult Neurogenesis in Brain Subventricular Zone

  • Luke L. Liu,
  • Richard M. van Rijn and
  • Wei Zheng

31 August 2022

The subventricular zone (SVZ) in lateral ventricles is the largest neurogenic region in adult brain containing high amounts of copper (Cu). This study aims to define the role of Cu in adult neurogenesis by chelating labile Cu ions using a well-establ...

  • Article
  • Open Access
7 Citations
4,836 Views
11 Pages

Synthesis and Characterization of Gold Chiral Nanoparticles Functionalized by a Chiral Drug

  • Simona Bettini,
  • Michela Ottolini,
  • Donato Valli,
  • Rosanna Pagano,
  • Chiara Ingrosso,
  • Maarten Roeffaers,
  • Johan Hofkens,
  • Ludovico Valli and
  • Gabriele Giancane

30 April 2023

Inorganic chiral nanoparticles are attracting more and more attention due to their peculiar optical properties and potential biological applications, such as bioimaging, therapeutics, and diagnostics. Among inorganic chiral nanoparticles, gold chiral...

  • Feature Paper
  • Article
  • Open Access
15 Citations
4,145 Views
18 Pages

The Presence of D-Penicillamine during the In Vitro Capacitation of Stallion Spermatozoa Prolongs Hyperactive-Like Motility and Allows for Sperm Selection by Thermotaxis

  • Sara Ruiz-Díaz,
  • Ivan Oseguera-López,
  • David De La Cuesta-Díaz,
  • Belén García-López,
  • Consuelo Serres,
  • Maria José Sanchez-Calabuig,
  • Alfonso Gutiérrez-Adán and
  • Serafin Perez-Cerezales

21 August 2020

Assisted reproductive technologies (ARTs) in the horse still yield suboptimal results in terms of pregnancy rates. One of the reasons for this is the lack of optimal conditions for the sperm capacitation in vitro. This study assesses the use of synth...

  • Article
  • Open Access
20 Citations
3,981 Views
11 Pages

Efficacy and Safety of Two Salts of Trientine in the Treatment of Wilson’s Disease

  • France Woimant,
  • Dominique Debray,
  • Erwan Morvan,
  • Mickael Alexandre Obadia and
  • Aurélia Poujois

8 July 2022

Background: Wilson’s disease (WD) is one of the few genetic disorders that can be successfully treated with pharmacological agents. Copper-chelating agents (D-penicillamine and Trientine salts) and zinc salts have been demonstrated to be effect...

  • Proceeding Paper
  • Open Access
1 Citations
1,693 Views
4 Pages

Sensing of Copper(II) by Immobilized Ligands: Comparison of Electrochemical and Surface Plasmon Resonance Transduction

  • Maria Pesavento,
  • Nunzio Cennamo,
  • Raffaela Biesuz,
  • Antonella Profumo and
  • Daniele Merli

Two sensors for the detection of the metal ion Cu(II), based on the same sensing layer, are compared. They rely on different transduction methods, i.e., electrochemistry and surface plasmon resonance (SPR). D,L-penicillamine was used as the specific...

  • Article
  • Open Access
1 Citations
1,526 Views
21 Pages

TDMQ20 as A Drug Candidate for Wilson’s Disease: Comparison with D-Penicillamine, Trientine, and Tetrathiomolybdate In Vitro and In Mice

  • Yingshan Zhu,
  • Weiling Peng,
  • Guangwei Liu,
  • Longxin Li,
  • Zikang Zhou,
  • Michel Nguyen,
  • Anne Robert,
  • Yan Liu and
  • Bernard Meunier

Background/Objectives: The lifelong treatment of Wilson’s disease (WD) currently relies on copper chelators with relatively poor metal specificity, which frequently exhibit serious adverse effects. There is a real medical need for a specific co...

  • Article
  • Open Access
20 Citations
7,994 Views
11 Pages

Polyvinyl chloride (PVC) is one of the most widely used polymers in medicine but has very poor biocompatibility when in contact with tissue or blood. To increase biocompatibility, controlled release of nitric oxide (NO) can be utilized to mitigate an...

  • Article
  • Open Access
3 Citations
2,596 Views
13 Pages

23 April 2023

A highly sensitive and accurate dual-signal strategy is developed for trace Cu2+ detection based on the inner filter effect (IFE) between Tween 20-gold nanoparticles (AuNPs) and CdSe/ZnS quantum dots (QDs). Tween 20-AuNPs are utilized as colorimetric...

  • Article
  • Open Access
10 Citations
2,820 Views
15 Pages

The Specific Copper(II) Chelator TDMQ20 Is Efficient for the Treatment of Wilson’s Disease in Mice

  • Yingshan Zhu,
  • Ying Tang,
  • Lan Huang,
  • Michel Nguyen,
  • Yan Liu,
  • Anne Robert and
  • Bernard Meunier

(1) Background: In patients with Wilson’s disease, the deficiency of the copper carrier ATP7B causes the accumulation of copper in the liver, brain and various other organs. Lifelong treatment is therefore mandatory, using copper chelators to i...

  • Article
  • Open Access
4 Citations
3,757 Views
8 Pages

Chiral Separation of rac-Propylene Oxide on Penicillamine Coated Gold NPs

  • Nisha Shukla,
  • Zachary Blonder and
  • Andrew J. Gellman

30 August 2020

The surfaces of chemically synthesized spherical gold NPs (Au-NPs) have been modified using chiral L- or D-penicillamine (Pen) in order to impart enantioselective adsorption properties. These chiral Au-NPs have been used to demonstrate enantioselecti...

  • Feature Paper
  • Article
  • Open Access
9 Citations
3,205 Views
15 Pages

Effects of Sodium Arsenite on the Myocardial Differentiation in Mouse Embryonic Bodies

  • SunHwa Jeong,
  • Changhwan Ahn,
  • Jin-Sook Kwon,
  • KangMin Kim and
  • Eui-Bae Jeung

1 February 2023

Arsenic in inorganic form is a known human carcinogen; even low levels of arsenic can interfere with the endocrine system. In mammalian development, arsenic exposure can cause a malformation of fetuses and be lethal. This study examined the effects o...

  • Feature Paper
  • Review
  • Open Access
17 Citations
6,468 Views
13 Pages

18 November 2022

The chelating thiol dimercaptosuccinate (DMSA) and the traditional agent D-penicillamine (PSH) are effective in enhancing the urinary excretion of copper (Cu) and lead (Pb) in poisoned individuals. However, DMSA, PSH, EDTA (ethylenediamine tetraaceta...

  • Article
  • Open Access
3 Citations
2,807 Views
10 Pages

CdS Nanoplates Modification as a Platform for Synthesis of Blue-Emitting Nanoparticles

  • Anna Lesiak,
  • Mateusz Banski,
  • Hanna Woznica,
  • Andrzej Żak,
  • Joanna Cabaj and
  • Artur Podhorodecki

In this paper, the study of surface modification of two-dimensional (2D), non-luminescent CdS nanoplates (NPLs) by thiol-containing ligands is presented. We show that a process of twophase transfers with appropriate ligand exchange transforms non-lum...

  • Article
  • Open Access
20 Citations
4,766 Views
17 Pages

Pigmentation Levels Affect Melanoma Responses to Coriolus versicolor Extract and Play a Crucial Role in Melanoma-Mononuclear Cell Crosstalk

  • Małgorzata Pawlikowska,
  • Tomasz Jędrzejewski,
  • Andrzej T. Slominski,
  • Anna A. Brożyna and
  • Sylwia Wrotek

Melanoma, the malignancy originating from pigment-producing melanocytes, is the most aggressive form of skin cancer and has a poor prognosis once the disease starts to metastasize. The process of melanin synthesis generates an immunosuppressive and m...

  • Case Report
  • Open Access
801 Views
9 Pages

Wilson’s Disease with Autoimmune Hepatitis Manifestation in a Pediatric Patient: A Case Report and Literature Review

  • Nicoleta Gimiga,
  • Gabriela Păduraru,
  • Laura-Iulia Bozomitu,
  • Gabriela Ghiga,
  • Monica Cristina Pânzaru,
  • Lăcrămioara Ionela Butnariu,
  • Ana Maria Scurtu,
  • Elena Cojocaru,
  • Vasile Valeriu Lupu and
  • Laura-Mihaela Trandafir

12 December 2025

Introduction: Wilson’s disease (WD) and autoimmune hepatitis (AIH) are important causes of acute and chronic hepatitis; each can lead to serious complications. The coexistence of these two diseases in the same patient is rare and poses signific...

  • Article
  • Open Access
4 Citations
2,418 Views
20 Pages

Copper-Induced Neurodegenerative Disorders and Therapeutic Potential of Curcumin-Loaded Nanoemulsion

  • Govind Hake,
  • Akshada Mhaske,
  • Rahul Shukla and
  • Swaran Jeet Singh Flora

29 January 2025

Copper accumulation in neurons induces oxidative stress, disrupts mitochondrial activity, and accelerates neuronal death, which is central to the pathophysiology of neurodegenerative diseases like Wilson disease. Standard treatments for copper toxici...

  • Article
  • Open Access
11 Citations
3,891 Views
15 Pages

Evaluation of Aminopolycarboxylate Chelators for Whole-Body Clearance of Free 225Ac: A Feasibility Study to Reduce Unexpected Radiation Exposure during Targeted Alpha Therapy

  • Mitsuyoshi Yoshimoto,
  • Yukie Yoshii,
  • Hiroki Matsumoto,
  • Mitsuhiro Shinada,
  • Masashi Takahashi,
  • Chika Igarashi,
  • Fukiko Hihara,
  • Tomoko Tachibana,
  • Ayano Doi and
  • Kohshin Washiyama
  • + 2 authors

Actinium-225 (225Ac) is a promising radionuclide used in targeted alpha therapy (TAT). Although 225Ac labeling of bifunctional chelating ligands is effective, previous in vivo studies reported that free 225Ac can be released from the drugs and that s...

  • Article
  • Open Access
10 Citations
3,167 Views
9 Pages

10 July 2023

Background: One of the most prevalent illnesses in neonates that needs care and treatment is neonatal jaundice. Several drugs are used as pharmacological modalities for treating hyperbilirubinemia, like intravenous immunoglobulin, D-penicillamine, me...

  • Review
  • Open Access
1 Citations
2,307 Views
18 Pages

23 December 2025

Chronic kidney disease (CKD) is a frequent and clinically significant comorbidity in patients with rheumatoid arthritis (RA), with a reported prevalence ranging from 20% to 50% depending on the cohort and definition applied. The high burden of CKD in...

  • Review
  • Open Access
7 Citations
2,669 Views
12 Pages

7 July 2022

Background: Wilson’s disease (WD) is an autosomal-recessive disorder of copper deposition caused by pathogenic variants in the copper-transporting ATP7B gene. There is not a clear correlation between genotype and phenotype in WD regarding sympt...

  • Article
  • Open Access
3 Citations
2,615 Views
16 Pages

Differences in the Time Course of Recovery from Brain and Liver Dysfunction in Conventional Long-Term Treatment of Wilson Disease

  • Harald Hefter,
  • Theodor S. Kruschel,
  • Max Novak,
  • Dietmar Rosenthal,
  • Tom Luedde,
  • Sven G. Meuth,
  • Philipp Albrecht,
  • Christian J. Hartmann and
  • Sara Samadzadeh

24 July 2023

Background: The aim of this study was to demonstrate that both neurological and hepatic symptoms respond to copper chelation therapy in Wilson disease (WD). However, the time course of their recovery is different. Methods: Eighteen patients with neur...

  • Review
  • Open Access
25 Citations
8,495 Views
39 Pages

Wilson’s Disease—Crossroads of Genetics, Inflammation and Immunity/Autoimmunity: Clinical and Molecular Issues

  • Grażyna Gromadzka,
  • Julia Czerwińska,
  • Elżbieta Krzemińska,
  • Adam Przybyłkowski and
  • Tomasz Litwin

20 August 2024

Wilson’s disease (WD) is a rare, autosomal recessive disorder of copper metabolism caused by pathogenic mutations in the ATP7B gene. Cellular copper overload is associated with impaired iron metabolism. Oxidative stress, cuproptosis, and ferrop...

  • Review
  • Open Access
93 Citations
12,351 Views
35 Pages

Wilson disease is a genetic disorder of the liver characterized by excess accumulation of copper, which is found ubiquitously on earth and normally enters the human body in small amounts via the food chain. Many interesting disease details were publi...

  • Article
  • Open Access
5 Citations
3,825 Views
19 Pages

Antifungal Effect of Penicillamine Due to the Selective Targeting of L-Homoserine O-Acetyltransferase

  • Aleksandra Kuplińska,
  • Kamila Rząd,
  • Marek Wojciechowski,
  • Sławomir Milewski and
  • Iwona Gabriel

Due to the apparent similarity of fungal and mammalian metabolic pathways, the number of established antifungal targets is low, and the identification of novel ones is highly desirable. The results of our studies, presented in this work, indicate tha...

  • Article
  • Open Access
4 Citations
2,553 Views
12 Pages

2 October 2022

A new chiral molecularly imprinted polymer (MIP) sensor with dual recognition ability was developed for the highly selective separation of enantiomers with toxic side effects in drugs. The sensor contains double-stranded deoxyribonucleic acid (dsDNA)...

  • Article
  • Open Access
4 Citations
2,497 Views
16 Pages

The Influence of Cationic Nitrosyl Iron Complex with Penicillamine Ligands on Model Membranes, Membrane-Bound Enzymes and Lipid Peroxidation

  • Darya A. Poletaeva,
  • Yuliya V. Soldatova,
  • Anastasiya V. Smolina,
  • Maxim A. Savushkin,
  • Elena N. Klimanova,
  • Nataliya A. Sanina and
  • Irina I. Faingold

2 November 2022

This paper shows the biological effects of cationic binuclear tetranitrosyl iron complex with penicillamine ligands (TNIC–PA). Interaction with a model membrane was assessed using a fluorescent probes technique. Antioxidant activity was studied...

  • Article
  • Open Access
43 Citations
6,591 Views
13 Pages

An Optical Fiber Chemical Sensor for the Detection of Copper(II) in Drinking Water

  • Maria Pesavento,
  • Antonella Profumo,
  • Daniele Merli,
  • Lucia Cucca,
  • Luigi Zeni and
  • Nunzio Cennamo

28 November 2019

Highly sensitive plasmonic optical fiber platforms combined with receptors have been recently used to obtain selective sensors. A low-cost configuration can be obtained exploiting a D-shaped plastic optical fiber covered with a multilayer sensing sur...

  • Article
  • Open Access
2 Citations
1,512 Views
9 Pages

28 June 2024

Chirality plays a significant part in many vital processes, and to further our level of understanding, there is a steadily growing interest in enhancing the yield of enantioselective processes. Here, a multilayer with etched grooves is activated in a...

  • Communication
  • Open Access
2,190 Views
9 Pages

Impact of Exogenous Nitric Oxide Treatment on Vascularization of a Subcutaneous Device for Cell Transplantation

  • Alexandra M. Smink,
  • Bryan Ceballos,
  • Taco Koster,
  • Samuel Rodriquez,
  • Michael Alexander,
  • Jonathan R. T. Lakey and
  • Paul de Vos

18 September 2022

Subcutaneous polymer scaffolds have shown potential for creating an optimal transplantation site in cellular replacement therapy, e.g., when transplanting insulin-producing cells to cure type 1 diabetes. Imperative for these scaffolds is a high degre...

  • Article
  • Open Access
31 Citations
4,291 Views
14 Pages

26 November 2021

Doxorubicin (Dox) is known to cause heart failure in some cancer patients. Despite extensive studies over the past half century, the subcellular basis of Dox-induced cardiomyopathy (DIC) is still elusive. Earlier, we suggested that Dox causes a delay...

  • Article
  • Open Access
7 Citations
4,306 Views
11 Pages

Contributions of Nitric Oxide to AHR-Ligand-Mediated Keratinocyte Differentiation

  • Carrie Hayes Sutter,
  • Haley M. Rainwater and
  • Thomas R. Sutter

Activation of the aryl hydrocarbon receptor (AHR) in normal human epidermal keratinocytes (NHEKs) accelerates keratinocyte terminal differentiation through metabolic reprogramming and reactive oxygen species (ROS) production. Of the three NOS isoform...

  • Article
  • Open Access
6 Citations
5,040 Views
11 Pages

7 December 2021

Metabolomics profiling using liquid chromatography-mass spectrometry (LC-MS) has become an important tool in biomedical research. However, resolving enantiomers still represents a significant challenge in the metabolomics study of complex samples. He...