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Structures of the human Wilson disease copper transporter ATP7B
Cell Reports, 2023 Summary: The P-type ATPase ATP7B exports cytosolic copper and plays an essential role in the regulation of cellular copper homeostasis. Mutants of ATP7B cause Wilson disease (WD), an autosomal recessive disorder of copper metabolism.
Guo-Min Yang +11 more
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Plumbagin Triggers Cuproptosis in Hepatocellular Carcinoma (HCC) via the DNA‐Methyltransferase 1 (DNMT1)/microRNA‐302a‐3p (miR‐302a‐3p)/ATPase Copper Transporting Beta (ATP7B) Axis [PDF]
MedCommInduction of cuproptosis in tumor cells is an emerging direction for cancer drug development. Plumbagin (PLB), a natural biological molecule, has anticancer activities, partially via copper‐dependent mechanisms.
Chuyu Wang +11 more
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BMC Medical Genomics, 2023 Background ATP7B is a copper-transporting protein that contributes to the chemo-resistance of human cancer cells. It remains unclear what the molecular mechanisms behind ATP7B are in cancer, as well as its role in human pan-cancer studies.
Zhanzhan Zhang +4 more
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Full-length ATP7B reconstituted through protein trans-splicing corrects Wilson disease in mice
Molecular Therapy: Methods & Clinical Development, 2022 Wilson disease (WD) is a genetic disorder of copper homeostasis, caused by deficiency of the copper transporter ATP7B. Gene therapy with recombinant adeno-associated vectors (AAV) holds promises for WD treatment. However, the full-length human ATP7B gene
Agnese Padula +14 more
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TFEB Regulates ATP7B Expression to Promote Platinum Chemoresistance in Human Ovarian Cancer Cells
Cells, 2022 ATP7B is a hepato-specific Golgi-located ATPase, which plays a key role in the regulation of copper (Cu) homeostasis and signaling. In response to elevated Cu levels, ATP7B traffics from the Golgi to endo-lysosomal structures, where it sequesters excess ...
Raffaella Petruzzelli +6 more
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PLoS Genetics, 2023 Copper (Cu) has a multifaceted role in brain development, function, and metabolism. Two homologous Cu transporters, Atp7a (Menkes disease protein) and Atp7b (Wilson disease protein), maintain Cu homeostasis in the tissue. Atp7a mediates Cu entry into the
Clorissa L Washington-Hughes +13 more
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Cells, 2021 Macroautophagy/autophagy plays an important role in cellular copper clearance. The means by which the copper metabolism and autophagy pathways interact mechanistically is vastly unexplored.
Supansa Pantoom +7 more
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Scientific Reports, 2021 Wilson disease (WD) is caused by inactivation of the copper transporter Atp7b and copper overload in tissues. Mice with Atp7b deleted either globally (systemic inactivation) or only in hepatocyte recapitulate various aspects of human disease.
Abigael Muchenditsi +10 more
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Cells, 2022 Copper is a transition metal essential for human life. Its homeostasis is regulated in the liver, which delivers copper to the whole body and excretes its excess outside the organism in the feces through the bile.
Peggy Charbonnier +5 more
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Direct Measurement of ATP7B Peptides Is Highly Effective in the Diagnosis of Wilson Disease
Gastroenterology, 2021 BACKGROUND AND AIMS Both existing clinical criteria and genetic testing have significant limitations for the diagnosis of Wilson's Disease (WD) often creating ambiguities in patient identification leading to delayed diagnosis and ineffective management ...
Christopher J. Collins +15 more
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