ArticleJournal of inherited metabolic disease2005
Effect of copper and diethyldithiocarbamate combination therapy on the macular mouse, an animal model of Menkes disease.
Article in Journal of inherited metabolic disease, 2005. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.
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Who cites it
16 citing papers in PubMed, 36 citations in OpenAlex.
- Exploring and Targeting the Connection of Iron and Copper Homeostasis to Neurodegenerative Diseases.MedComm · 2026Review
- Cuproptosis: unveiling a new frontier in cancer biology and therapeutics.Cell communication and signaling : CCS · 2024Review
- Decreased Expression of theInternational journal of molecular sciences · 2022Article
- Early clinical signs and treatment of Menkes disease.Molecular genetics and metabolism reports · 2022Article
- Pharmacokinetics of CuGTSM, a Novel Drug Candidate, in a Mouse Model of Menkes Disease.Pharmaceutical research · 2021Article
- Clinical and Molecular Delineation of Cutis Laxa Syndromes: Paradigms for Homeostasis.Advances in experimental medicine and biology · 2021Review
- Menkes disease: Oral administration of glyoxal-bis(N(4)-methylthiosemicarbazonato)-copper(II) rescues the macular mouse.Pediatric research · 2018Article
- Disulfiram enhanced delivery of orally administered copper into the central nervous system in Menkes disease mouse model.Journal of inherited metabolic disease · 2018Article
- Transcriptome Sequencing Reveals Candidate NF-κB Target Genes Involved in Repeated Cocaine Administration.The international journal of neuropsychopharmacology · 2018Article
- Drosophila melanogaster Models of Metal-Related Human Diseases and Metal Toxicity.International journal of molecular sciences · 2017Review
- The Activity of Menkes Disease Protein ATP7A Is Essential for Redox Balance in Mitochondria.The Journal of biological chemistry · 2016Article
- Menkes disease: what a multidisciplinary approach can do.Journal of multidisciplinary healthcare · 2016Review
- Pharmacological activity of metal binding agents that alter copper bioavailability.Dalton transactions (Cambridge, England : 2003) · 2015Article
- Mottled Mice and Non-Mammalian Models of Menkes Disease.Frontiers in molecular neuroscience · 2015Review
- Inherited copper transport disorders: biochemical mechanisms, diagnosis, and treatment.Current drug metabolism · 2012Review
- Article
Corrections and comments
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Authors and funding
6 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Menkes disease (MD) is a neurodegenerative disorder characterized by a copper deficiency in the brain. It is caused by the defective intestinal absorption of copper resulting from a deficiency of a copper-transporting ATPase, ATP7A. This gives rise to an accumulation of copper in the intestine. The copper deficiency in the brain of MD patients cannot be improved by copper injections, because the administered copper accumulates at the blood-brain barrier and is not transported across to the neurons. To resolve this problem, we investigated the effect of a combination therapy of copper and sodium diethyldithiocarbamate (DEDTC), a lypophilic chelator, in an animal model of MD, the macular mouse. Four-week-old macular mice treated with 50 mug of CuCl2 on the 7th day after birth were used. Experimental mice were given a subcutaneous injection of CuCl2 (4 microg) and an intraperitoneal injection of DEDTC (0.2 mg/g body weight) twice a week for 4 weeks and then sacrificed. Copper concentrations and cytochrome-c oxidase activity in the brains of treated mice were higher than those of control macular mice, which received only copper or saline. The ratios of brain noradrenaline to dopamine and of adrenaline to dopamine were also increased by the treatment, suggesting that the activity of dopamine beta-hydroxylase, a copper-dependent enzyme, was improved by the treatment. Liver and renal function tests showed no abnormalities in the treated mice, although copper concentrations in the kidneys of treated mice were higher than those of control macular mice. These results suggest that DEDTC facilitates the passage of copper across the blood-brain barrier and that the combination therapy of copper and DEDTC may be an effective treatment for the neurological disturbances suffered by patients with MD.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.