Metal-Dependent Regulation of ATP7A and ATP7B in Fibroblast Cultures
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چکیده
منابع مشابه
Metal-Dependent Regulation of ATP7A and ATP7B in Fibroblast Cultures
Deficiency of one of the copper transporters ATP7A and ATP7B leads to the rare X-linked disorder Menkes Disease (MD) or the rare autosomal disorder Wilson disease (WD), respectively. In order to investigate whether the ATP7A and the ATP7B genes may be transcriptionally regulated, we measured the expression level of the two genes at various concentrations of iron, copper, and insulin. Treating f...
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Rachel Linz,* Natalie L. Barnes,* Adriana M. Zimnicka, Jack H. Kaplan, Betty Eipper, and Svetlana Lutsenko Department of Biochemistry and Molecular Biology, Oregon Health and Science University, Portland, Oregon; Department of Biochemistry and Molecular Genetics, University of Illinois, Chicago, Illinois; and Department of Neuroscience, University of Connecticut Health Center, Farmington, Conne...
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Over the past two decades there have been significant advances in our understanding of copper homeostasis and the pathological consequences of copper dysregulation. Cumulative evidence is revealing a complex regulatory network of proteins and pathways that maintain copper homeostasis. The recognition of copper dysregulation as a key pathological feature in prominent neurodegenerative disorders ...
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ATP7A and ATP7B are P-type ATPases required for copper homeostasis and involved in the etiology of Menkes and Wilson diseases. We used heterologous expression of ATP7A or ATP7B in COS-1 cells infected with adenovirus vectors to characterize differential features pertinent to each protein expressed in the same mammalian cell type, rather than to extrinsic factors related to different cells susta...
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Kidneys regulate their copper content more effectively than many other organs in diseases of copper deficiency or excess. We demonstrate that two copper-transporting ATPases, ATP7A and ATP7B, contribute to this regulation. ATP7A is expressed, to a variable degree, throughout the kidney and shows age-dependent intracellular localization. In 2-wk-old mice, ATP7A is located in the vicinity of the ...
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ژورنال
عنوان ژورنال: Frontiers in Molecular Neuroscience
سال: 2016
ISSN: 1662-5099
DOI: 10.3389/fnmol.2016.00068