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A hierarchic gene expression of copper homeostatic genes was demonstrated between atp7a, sp1 (show SP1 Proteins) and sod1 (show SOD1 Proteins) in zebrafish.
The investigators interrogated a genetic screen for embryos phenocopied by copper deficiency, identifying calamity, a mutant defective in the zebrafish ortholog of the Menkes disease gene (atp7a).
The P-type copper ATPases ATP7A and ATP7B (show ATP7B Proteins) provide an important system for acquisition, active transport, distribution and elimination of copper. Relevance of copper metabolism to human diseases and therapy is already known. It is quite certain that further studies will reveal detailed and useful information on biochemical mechanisms and relevance to diseases
the mechanism of copper-dependent regulation of ATP7B (show ATP7B Proteins) and ATP7A, the roles of individual MBDs, and the relationship between the regulatory and catalytic copper binding are still unknown. We describe the structure and dynamics of the MBDs, review the current knowledge about their functional roles and propose a mechanism of regulation of ATP7B (show ATP7B Proteins) by copper-dependent changes in the dynamics and conformation of the MBD (show DPEP1 Proteins) chain.
Deletion spanning exons 8 to 12 of the ATP7A gene is associated with a family affected with Menkes disease.
ATP-dependent copper transfer in ATP7A/B is not affected by varying the pH, suggesting that net proton counter-transport may not occur in copper ATPases. Platinum anticancer drugs activate ATP7A/B and are subjected to ATP-dependent vectorial displacement with a mechanism analogous to that of copper
studies show that merestinib effectively blocks eIF4E (show EIF4E Proteins) phosphorylation in AML (show RUNX1 Proteins) cells and suppresses primitive leukemic progenitors from AML (show RUNX1 Proteins) patients in vitro and in an AML (show RUNX1 Proteins) xenograft model in vivo.
It has been demonstrated in ovarian cancer cells that cisplatin resistance and uptake correlates with reduced CTR1 (show SLC31A1 Proteins) and LRRC8A (show LRRC8A Proteins) protein expression/activity and a concomitant upregulation in cisplatin exporting transporters (ATP7A, ATP7B (show ATP7B Proteins)), which implies that the resistant cells have a reduced ability to accumulate cisplatin and activate proapoptotic transporters for osmolytes.
Thus, ATP7A activity protects mitochondria from excessive copper entry, which is deleterious to redox buffers. Mitochondrial redox misbalance could significantly contribute to pathologies associated with ATP7A inactivation in tissues with paradoxical accumulation of copper
Cu chaperone function of Atox1 (show ATOX1 Proteins) mediated through Cu transporter ATP7A is required for VEGF (show VEGFA Proteins)-induced angiogenesis via activation of Cu enzyme lysyl oxidase (show LOX Proteins).
Upon neuronal differentiation, transitions in redox states upregulates expression of ATOX1 (show ATOX1 Proteins) and its partner ATP7A, producing higher flux of copper through the secretory pathway.
CTR1 (show SLC31A1 Proteins), ATP7A, and lysyl oxidase (show LOX Proteins) were upregulated in the lung tissues and pulmonary arteries of mice with hypoxia-induced pulmonary hypertension and pulmonary arterial smooth muscle cells.
study characterized the Mottled-dappled deletion in C3H101H carrier females
Results from experiments in mouse model of Menkes disease harboring ATP5a (show ATP5G1 Proteins) mutation, show increase expression of SOD1 (show SOD1 Proteins) and induction of HO-1 (show HMOX1 Proteins) causing iron metabolism disruption and hemolysis due to copper deficiency.
The MPhi ATP7A selectively regulates LPS (show TLR4 Proteins)-induced inflammatory mediators, in part, via modulation of cellular copper availability, whereas neocuproine generally inhibits the production of inflammatory mediators.
a decrease in ATP7A protein expression contributes to impaired SOD3 (show SOD3 Proteins) activity, resulting in O2(*-) overproduction and endothelial dysfunction in blood vessels of type 1 diabetes mellitus.
ATP7A mutations leading to Menkes disease and occipital horn syndrome in human and animal models [Review]
These studies demonstrate the essential role of the Atp7a gene in mouse embryonic development and establish a powerful model for understanding the tissue-specific roles of ATP7A in copper metabolism and disease.
ATP7A-mediated copper homeostasis is important for the formation of pathogenic proteinase-resistant prion protein (show PRNP Proteins)
This gene encodes a transmembrane protein that functions in copper transport across membranes. This protein is localized to the trans-Golgi network, where it is predicted to supply copper to copper-dependent enzymes in the secretory pathway. It relocalizes to the plasma membrane under conditions of elevated extracellular copper, and functions in the efflux of copper from cells. Mutations in this gene are associated with Menkes disease, X-linked cutis laxa, and occipital horn syndrome.
ATPase, Cu++ transporting, alpha polypeptide (Menkes syndrome)
, ATPase, Cu++ transporting, alpha polypeptide
, Menkes disease ATPase
, copper-transporting ATPase 1
, copper-transporting P-type ATPase
, copper-transporting ATPase
, ATPase protein
, Copper-transporting ATPase 1
, copper-transporting ATPase 1-like
, Cu++-transporting P-type ATPase
, Menkes disease-associated protein
, copper pump 1
, Menkes protein
, menkes disease-associated protein homolog
, copper transporting ATPase