Treatment of Wilson's disease with tetrathiomolybdate: V. control of free copper by tetrathiomolybdate and a comparison with trientine

Brewer, George J.; Askari, Fred; Dick, Robert; Sitterly, Julia; Fink, John K.; Carlson, Martha; Kluin, Karen J.; Lorincz, Matthew T.

doi:10.1016/j.trsl.2009.05.002

Cited by 162 publications

(143 citation statements)

References 17 publications

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“…As tetrathiomolybdate is commercially unavailable so zinc therapy is recommended. 2,9 This case series shows that phenotypic variability of WD was very high and even different patients with same genetic makeup (siblings) of a family may present with its diverse manifestations.…”

Section: Discussionmentioning

confidence: 87%

Varied and atypical presentation of Wilson disease in a family: report of 3 cases in siblings

Ranjan

Kumar

2016

Int J Contemp Pediatr

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Section: Discussionmentioning

confidence: 87%

Varied and atypical presentation of Wilson disease in a family: report of 3 cases in siblings

Ranjan

Kumar

2016

Int J Contemp Pediatr

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“…The risk of initial neurological worsening could be lessened with tetrathiomolybdate, another copper chelator, which acts by forming a tripartite complex with copper and protein either in the intestinal lumen to prevent copper absorption, or in the circulation where it blocks cellular copper trafficking [18]. However, clinical experience with this drug remains insufficient and international studies are still only in the pipeline.…”

Section: Wilson's Disease (Wd)mentioning

confidence: 99%

Bioavailable Trace Metals in Neurological Diseases

Poujois

Devedjian

Moreau

et al. 2016

Curr Treat Options Neurol

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Opinion statement  Medical treatment in Wilson's disease includes chelators (D-Penicillamine andTrientine) or zinc salts that have to be maintain all the lifelong. This pharmacological treatment is categorized into two phases; the first being a decoppering phase and the second a maintenance's one. The best therapeutic approach remains controversial, as only a few non-controlled trials have compared these treatments. During the initial phase, progressive increase of chelators' doses adjusted to exchangeable copper and urinary copper might help to avoid neurological deterioration. Liver transplantation is indicated in acute fulminant liver failure and decompensated cirrhosis; in cases of neurologic deterioration, it must be individually discussed. During the maintenance phase, the most important challenge is to obtain a good adherence to lifelong medical therapy. Neurodegenerative diseases that lead to a mislocalization of iron can be caused by a culmination of localized overload (pro-oxidant siderosis) and localized deficiency (metabolic distress). A new therapeutic concept with conservative iron chelation rescues iron-overloaded neurons by scavenging labile iron and, by delivering this chelated metal to endogenous apotransferrin, allows iron redistribution to avoid systemic loss of iron.

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“…It is also used in the clinical setting to treat Wilson disease with neurological manifestations. [87][88][89][90][91] Cu that accumulates in the liver of Wilson disease patients and their animal models, such as LEC rats, is predominantly bound to MT. The affinity of Cu for MT is known to be too high, rendering both penicillamine and Fig.…”

Section: Cu Chelatorsmentioning

confidence: 99%

Research Tools and Techniques for Copper Metabolism in Mammals

Ogra

2011

JOURNAL OF HEALTH SCIENCE

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Copper (Cu) is an essential component of biological redox reactions and its deficiency is fatal to the body. At the same time, Cu is extremely toxic when present in excess. In this regard, several groups of Cu-regulating proteins in the body act to regulate the concentration of Cu within a certain range. However, the overall mechanism underlying the maintenance of Cu homeostasis in the body and cells remains poorly understood. In this review, recent research tools, such as animal models and gene-modified animals, and techniques, such as speciation and imaging of Cu, are highlighted.

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Treatment of Wilson's disease with tetrathiomolybdate: V. control of free copper by tetrathiomolybdate and a comparison with trientine

Cited by 162 publications

References 17 publications

Varied and atypical presentation of Wilson disease in a family: report of 3 cases in siblings

Varied and atypical presentation of Wilson disease in a family: report of 3 cases in siblings

Bioavailable Trace Metals in Neurological Diseases

Research Tools and Techniques for Copper Metabolism in Mammals

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