ArticleAmerican journal of physiology. Gastrointestinal and liver physiology2012
Maternofetal and neonatal copper requirements revealed by enterocyte-specific deletion of the Menkes disease protein.
Article in American journal of physiology. Gastrointestinal and liver physiology, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 27 papers.
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Who cites it
27 citing papers in PubMed, 39 citations in OpenAlex.
- Emerging role of copper in the pathophysiology of spinal cord injury.Neural regeneration research · 2026Article
- Research progress on cuproptosis and copper related anti-tumor therapy.Discover oncology · 2025Review
- Adaptive protein synthesis in genetic models of copper deficiency and childhood neurodegeneration.Molecular biology of the cell · 2025Article
- Adaptive protein synthesis in genetic models of copper deficiency and childhood neurodegeneration.bioRxiv : the preprint server for biology · 2024Article
- Association between dietary copper intake and constipation in US adults.Scientific reports · 2024Article
- The physiological and pathophysiological roles of copper in the nervous system.The European journal of neuroscience · 2024Review
- Pigmentation and TYRP1 expression are mediated by zinc through the early secretory pathway-resident ZNT proteins.Communications biology · 2023Article
- Connecting copper and cancer: from transition metal signalling to metalloplasia.Nature reviews. Cancer · 2022Review
- Oral Elesclomol Treatment Alleviates Copper Deficiency in Animal Models.Frontiers in cell and developmental biology · 2022Article
- Copper Homeostatic Mechanisms and Their Role in the Virulence of Escherichia coli and Salmonella enterica.EcoSal Plus · 2021Review
- Dynamic and cell-specific transport networks for intracellular copper ions.Journal of cell science · 2021Review
- Copper metabolism as a unique vulnerability in cancer.Biochimica et biophysica acta. Molecular cell research · 2021Review
- Review
- Adipocyte-specific disruption of ATPase copper transporting α in mice accelerates lipoatrophy.Diabetologia · 2019Article
- Copper Transport and Disease: What Can We Learn from Organoids?Annual review of nutrition · 2019Review
- Trafficking mechanisms of P-type ATPase copper transporters.Current opinion in cell biology · 2019Review
- The Function of ATPase Copper Transporter ATP7B in Intestine.Gastroenterology · 2018Article
- Dietary Copper Reduces the Hepatotoxicity of (-)-Epigallocatechin-3-Gallate in Mice.Molecules (Basel, Switzerland) · 2017Article
- Copper trafficking to the secretory pathway.Metallomics : integrated biometal science · 2016Review
- Knockdown of copper-transporting ATPase 1 (Atp7a) impairs iron flux in fully-differentiated rat (IEC-6) and human (Caco-2) intestinal epithelial cells.Metallomics : integrated biometal science · 2016Article
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Authors and funding
7 authors at 3 institutions in 1 country.
Funding
Abstract
The essential requirement for copper in early development is dramatically illustrated by Menkes disease, a fatal neurodegenerative disorder of early childhood caused by loss-of-function mutations in the gene encoding the copper transporting ATPase ATP7A. In this study, we generated mice with enterocyte-specific knockout of the murine ATP7A gene (Atp7a) to test its importance in dietary copper acquisition. Although mice lacking Atp7a protein within intestinal enterocytes appeared normal at birth, they exhibited profound growth impairment and neurological deterioration as a consequence of copper deficiency, resulting in excessive mortality prior to weaning. Copper supplementation of lactating females or parenteral copper injection of the affected offspring markedly attenuated this rapid demise. Enterocyte-specific deletion of Atp7a in rescued pregnant females did not restrict embryogenesis; however, copper accumulation in the late-term fetus was severely reduced, resulting in early postnatal mortality. Taken together, these data demonstrate unique and specific requirements for enterocyte Atp7a in neonatal and maternofetal copper acquisition that are dependent on dietary copper availability, thus providing new insights into the mechanisms of gene-nutrient interaction essential for early human development.
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