Results 221 to 230 of about 5,675 (258)

Insights into the mechanism of copper transport by the Wilson and Menkes disease copper-transporting ATPases

Inorganica Chimica Acta, 2002
Menkes and Wilson diseases are two closely related hereditary disorders of copper metabolism. The genes for these disorders have been cloned and identified as copper-transporting ATPases, which are members of a large family of cation transporters, the P-type ATPases.
Negah Fatemi, Bibudhendra Sarkar
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The Copper-Transporting ATpase Defective in Wilson Disease

1999
Two critical genes have been recognized as essential for the export of copper from cells. The proteins encoded by these genes are very similar, yet perform different functions. Between them, they play a role in controlling copper levels in cells, providing for copper essential for number of enzymes, while preventing the accumulation of toxic levels of ...
Diane W. Cox, John R. Forbes, Manoj S. Nanji   +2 more
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A copper-transporting ATPase BcCCC2 is necessary for pathogenicity of Botrytis cinerea

Molecular Genetics and Genomics, 2010
Copper is an essential trace element that serves as a cofactor for numerous enzymes. In eukaryotes, copper-transporting ATPases deliver copper to various copper-containing proteins in the trans-golgi network. This study identified a copper-transporting ATPase gene BcCcc2 in a fungus pathogenic to plants, Botrytis cinerea. We investigated the biological
Yoshimoto, Saitoh, Kosuke, Izumitsu, Atsushi, Morita, Chihiro, Tanaka   +3 more
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Membrane Structure of CtrA3, a Copper-transporting P-type-ATPase from Aquifex aeolicus

Journal of Molecular Biology, 2008
We have produced and characterized two new copper-transporting ATPases, CtrA2 and CtrA3 from Aquifex aeolicus, that belong to the family of heavy metal ion-transporting P(IB)-type ATPases. CtrA2 has a CPC metal-binding sequence in TM6 and a CxxC metal-binding N-terminal domain, while CtrA3 has a CPH metal-binding motif in TM6 and a histidine-rich N ...
Chintalapati, S., Al Kurdi, R., Terwisscha van Scheltinga, A., Kühlbrandt, W. ; https://orcid.org/0000-0002-2013-4810   +3 more
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Copper Does Not Alter the Intracellular Distribution of ATP7B, a Copper-Transporting ATPase

Biochemical and Biophysical Research Communications, 2000
Wilson's disease is a genetic disorder characterized by the accumulation of copper in the body due to a defect of biliary copper excretion. However, the mechanism of biliary copper excretion has not been fully clarified. We examined the effect of copper on the intracellular localization of the Wilson disease gene product (ATP7B) and green fluorescent ...
M, Harada, S, Sakisaka, T, Kawaguchi, R, Kimura, E, Taniguchi, H, Koga, S, Hanada, S, Baba, K, Furuta, R, Kumashiro, T, Sugiyama, M, Sata   +11 more
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Human Copper-Transporting ATPase ATP7B (The Wilson's Disease Protein): Biochemical Properties and Regulation

Journal of Bioenergetics and Biomembranes, 2002
Wilson's disease protein (WNDP) is a product of a gene ATP7B that is mutated in patients with Wilson's disease, a severe genetic disorder with hepatic and neurological manifestations caused by accumulation of copper in the liver and brain. In a cell, WNDP transports copper across various cell membranes using energy of ATP-hydrolysis.
Svetlana, Lutsenko, Roman G, Efremov, Ruslan, Tsivkovskii, Joel M, Walker   +3 more
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Phosphorylation regulates copper-responsive trafficking of the Menkes copper transporting P-type ATPase

The International Journal of Biochemistry & Cell Biology, 2009
The Menkes copper-translocating P-type ATPase (ATP7A) is a critical copper transport protein functioning in systemic copper absorption and supply of copper to cuproenzymes in the secretory pathway. Mutations in ATP7A can lead to the usually lethal Menkes disease.
Veldhuis, Nicholas A., Valova, Valentina A., Gaeth, Ann P., Palstra, Nickless, Hannan, Katherine M., Michell, Belinda J., Kelly, Leonard E., Jennings, Ian, Kemp, Bruce E., Pearson, Richard B., Robinson, Phillip J., Camakaris, James   +11 more
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[From gene to disease: copper-transporting P ATPases alteration].

Pathologie-biologie, 2009
Copper is a trace metal, essential for many biological processes. Copper is also toxic if in excessive amounts; its homeostatic balance requires a delicate regulation. Several severe hereditary human disorders of copper regulatory mechanisms have been identified; they are related to mutations in gene ATP7A and ATP7B coding for copper-transporting ...
C, Garcia Hejl, C, Vrignaud, C, Garcia, F, Ceppa   +3 more
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Phylogenetic analysis of heavy-metal ATPases in fungi and characterization of the copper-transporting ATPase of Cochliobolus heterostrophus

Mycological Research, 2009
We performed a phylogenetic analysis of heavy-metal ATPases (HMAs) in fungi and found that HMAs can be divided into three groups, A, B, and C. Group A is predicted to deliver copper ions to copper-containing proteins, while Groups B and C are thought to function as cell-membrane copper-efflux pumps.
Yoshimoto, Saitoh, Kosuke, Izumitsu, Chihiro, Tanaka   +2 more
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Mutants in the CtpA copper transporting P-type ATPase reduce virulence ofListeria monocytogenes

Microbial Pathogenesis, 1997
The CtpA protein from pathogenic Listeria monocytogenes, is a P-type adenosine triphosphatase involved in copper homeostasis. To establish a role in pathogenicity for CtpA, a mutant strain was constructed by insertion of an antibiotic resistance cartridge into the ctpA gene.
Francis, Matthew S., Thomas, Connor J.
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