Exploring the potential of trientine tetrahydrochloride in the treatment of Wilson disease

Sharma, Nayan; Das, Debashree Debasish; Chawla, Pooja

doi:10.1016/j.hsr.2023.100082

“…Triethylenetetramine (TETA), also known as trientine, is in patients who have severe intolerance to D-penicillin, this agent is considered an oral chelating agent and has less adverse effects. Evidence based on reduction of body copper stores of "decoppered" and improvement of clinical symptoms was reported by Trientine [63]. TETA causes copper to be excreted in the urine and studies have shown it to be safer.…”

Section: Rhabdomyolysismentioning

confidence: 99%

Copper Poisoning with Emphasis on Its Clinical Manifestations and Treatment of Intoxication

Rafati Rahimzadeh,

Kazemi

et al. 2024

Advances in Public Health

2

0

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Background. Copper is an essential trace element found in the human body in an oxidized (Cu II) and reduced (Cu I) form. It plays a crucial role in the integrity and function of proteins and enzymes. Short-term and long-term exposure to copper can result in harmful effects and lead to clinical manifestations in multiple bodily systems, including the gastrointestinal tract, liver, kidneys, eyes, respiratory, cardiovascular, central nervous, endocrine, and hematopoietic systems. Objective. The purpose of this study is the importance of early recognition and diagnosis of copper poisoning immediate and necessary measures and the use of chelators. Materials and Methods. In this review article, authors from Pub Med, Scopus, and a toxicologic emergencies reference book from 1996 to 2024 are used. Result. An excessive increase in copper level produces reactive oxygen species that can cause lipid peroxidation in cell membranes, direct oxidation of proteins, and the breakdown of DNA and RNA molecules. All of these can generally be reasons for cell death. Conclusion. Assessing levels of copper in whole blood, free serum copper, 24-hr urine copper, liver biopsy for copper concentration, and ceruloplasmin play a crucial role in the diagnosis. The blood copper concentration is directly related to the severity of poisoning. Treatment for copper poisoning typically involves removing the source of exposure and administering medications to help remove the excess copper from the body. Supportive care for copper intoxication usually includes managing vomiting, correcting fluids and electrolytes, and stabilizing vital signs. Chelators like D-penicillamine, succimer, trientine, tetrathiomolybdate, and PBT-2 have been utilized in treatment.

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“…CBD is popularly used for anti-cancer [58] due to its anti-chemoresistance, redox, and biocompatibility properties [71][72]. For instance, the common oral administrations used for Wilson disease are d-penicillamine, tetrathiomolybdate, and triethylene tetramine [73]. In this therapy, the copper chelator binds the excess copper to maintain genetic homeostasis [74].…”

Section: Copper and Zinc For Anti-chemoresistance In Osteosarcoma The...mentioning

confidence: 99%

“…In this therapy, the copper chelator binds the excess copper to maintain genetic homeostasis [74]. Because of the urine and biliary excretions, the outcomes of this therapy are low toxicity, fewer side effects, and easy diagnosis [73,75]. As a result, this chelator modulates homeostasis by regulating the expression of high-affinity copper uptake protein (CTR)-1 [76].…”

Section: Copper and Zinc For Anti-chemoresistance In Osteosarcoma The...mentioning

confidence: 99%

Combining Copper and Zinc into a Biosensor for Anti-Chemoresistance and Achieving Osteosarcoma Therapeutic Efficacy

Zaidi¹,

Miskon²

2023

Preprint

0

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Due to its built-up chemoresistance after prolonged usage, the demand for replacing platinum in metal-based drugs (MBD) is rising. The first MBD approved by the FDA for cancer therapy was cisplatin in 1978. Even after nearly four and a half decades of trials, there has been no significant improvement in osteosarcoma (OS) therapy. In fact, many MBD have been developed, but the chemoresistance problem raised by platinum remains unresolved. This motivates us to elucidate the possibilities of the copper and zinc (CuZn) combination to replace platinum in MBD. Thus, the anti-chemoresistance properties of CuZn and their physiological functions for OS therapy are highlighted. Herein, we summarise their chelators, main organic solvents, and ligand functions in their structures that are involved in anti-chemoresistance properties. Through this review, it is rational to discuss their ligands’ roles as biosensors in drug delivery systems. Hereafter, an in-depth understanding of their redox and photoactive function relationships is provided. The disadvantage is that the other functions of biosensors cannot be elaborated on here. As a result, this review is being developed, which is expected to intensify OS drugs with higher cure rates. Nonetheless, this advancement intends to solve the major chemoresistance obstacle towards clinical efficacy.

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“…CBD is popularly used for anti-cancer [ 55 ] due to its anti-chemoresistance, redox, and biocompatibility properties [ 68 , 69 ]. For instance, the common oral administrations used for Wilson’s disease are d-penicillamine, tetrathiomolybdate, and triethylene tetramine [ 70 ]. In this therapy, the copper chelator binds the excess copper to maintain genetic homeostasis [ 71 ].…”

Section: Copper and Zinc For Anti-chemoresistance In Osteosarcoma The...mentioning

confidence: 99%

“…In this therapy, the copper chelator binds the excess copper to maintain genetic homeostasis [ 71 ]. Due to the urine and biliary excretions, the outcomes of this therapy are low toxicity, fewer side effects, and easy diagnosis [ 70 , 72 ]. As a result, this chelator modulates homeostasis by regulating the expression of high-affinity copper uptake protein (CTR)-1 [ 73 ].…”

Section: Copper and Zinc For Anti-chemoresistance In Osteosarcoma The...mentioning

confidence: 99%

Combining Copper and Zinc into a Biosensor for Anti-Chemoresistance and Achieving Osteosarcoma Therapeutic Efficacy

Zaidi

¹

,

Miskon

²

2023

Molecules

17

0

View full text Add to dashboard Cite

Due to its built-up chemoresistance after prolonged usage, the demand for replacing platinum in metal-based drugs (MBD) is rising. The first MBD approved by the FDA for cancer therapy was cisplatin in 1978. Even after nearly four and a half decades of trials, there has been no significant improvement in osteosarcoma (OS) therapy. In fact, many MBD have been developed, but the chemoresistance problem raised by platinum remains unresolved. This motivates us to elucidate the possibilities of the copper and zinc (CuZn) combination to replace platinum in MBD. Thus, the anti-chemoresistance properties of CuZn and their physiological functions for OS therapy are highlighted. Herein, we summarise their chelators, main organic solvents, and ligand functions in their structures that are involved in anti-chemoresistance properties. Through this review, it is rational to discuss their ligands’ roles as biosensors in drug delivery systems. Hereafter, an in-depth understanding of their redox and photoactive function relationships is provided. The disadvantage is that the other functions of biosensors cannot be elaborated on here. As a result, this review is being developed, which is expected to intensify OS drugs with higher cure rates. Nonetheless, this advancement intends to solve the major chemoresistance obstacle towards clinical efficacy.

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Exploring the potential of trientine tetrahydrochloride in the treatment of Wilson disease

Cited by 5 publications

References 107 publications

Copper Poisoning with Emphasis on Its Clinical Manifestations and Treatment of Intoxication

Copper Poisoning with Emphasis on Its Clinical Manifestations and Treatment of Intoxication

Combining Copper and Zinc into a Biosensor for Anti-Chemoresistance and Achieving Osteosarcoma Therapeutic Efficacy

Combining Copper and Zinc into a Biosensor for Anti-Chemoresistance and Achieving Osteosarcoma Therapeutic Efficacy

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