Results 211 to 220 of about 5,675 (258)

Role and mechanisms of cuproptosis in the pathogenesis of Wilson's disease (Review). [PDF]

Int J Mol Med
Chen H, Wang X, Xing J, Pu Y, Ye H, Ma Y, Zhang J.   +6 more
europepmc   +1 more source

From severe aplastic anemia with TERT variant to Wilson disease - associations or not. [PDF]

Ann Hematol
Chen T, Song J, Xing L, Chen J, Dong X, Li L, Yang J, Liu W, Shao Z, Fu R.   +9 more
europepmc   +1 more source

Copper Trafficking in Caenorhabditis elegans : Copper Chaperone and Copper Transporting ATPase

Copper Trafficking in Caenorhabditis elegans : Copper Chaperone and Copper Transporting ATPase
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Function and Regulation of Human Copper-Transporting ATPases

Physiological Reviews, 2007
Copper-transporting ATPases (Cu-ATPases) ATP7A and ATP7B are evolutionarily conserved polytopic membrane proteins with essential roles in human physiology. The Cu-ATPases are expressed in most tissues, and their transport activity is crucial for central nervous system development, liver function, connective tissue formation, and many other ...
Svetlana, Lutsenko, Natalie L, Barnes, Mee Y, Bartee, Oleg Y, Dmitriev   +3 more
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Copper Transporting P-Type ATPases and Human Disease

Journal of Bioenergetics and Biomembranes, 2002
Copper transporting P-type ATPases, designated ATP7A and ATP7B, play an essential role in mammalian copper balance. Impaired intestinal transport of copper, resulting from mutations in the ATP7A gene, lead to Menkes disease in humans. Defects in a similar gene, the copper transporting ATPase ATP7B, result in Wilson disease.
Diane W, Cox, Steven D P, Moore
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Conservation of copper-transporting P(IB)-type ATPase function

BioMetals, 2010
Copper-transporting P(IB)-type ATPases are highly conserved, and while unicellular eukaryotes and invertebrates have only one, a gene duplication has occurred during vertebrate evolution. Copper-induced trafficking of mammalian ATP7A and ATP7B from the trans-Golgi Network towards the plasma membrane is critical for their role in copper homeostasis.
Southon, Adam, Palstra, Nickless, Veldhuis, Nicholas, Gaeth, Ann, Robin, Charles, Burke, Richard, Camakaris, James   +6 more
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Copper-transporting ATPases ATP7A and ATP7B: cousins, not twins

Journal of Bioenergetics and Biomembranes, 2007
Copper plays an essential role in human physiology and is indispensable for normal growth and development. Enzymes that are involved in connective tissue formation, neurotransmitter biosynthesis, iron transport, and others essential physiological processes require copper as a cofactor to mediate their reactions. The biosynthetic incorporation of copper
Rachel, Linz, Svetlana, Lutsenko
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Structural and Functional Insights of Wilson Disease Copper-Transporting ATPase

Journal of Bioenergetics and Biomembranes, 2002
Wilson disease is an autosomal recessive disorder of copper metabolism. The gene for this disorder has been cloned and identified to encode a copper-transporting ATPase (ATP7B), a member of a large family of cation transporters, the P-type ATPases. In addition to the core elements common to all P-type ATPases, the Wilson copper-transporting ATPase has ...
Negah, Fatemi, Bibudhendra, Sarkar
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Copper ATPase CopA from Escherichia coli: Quantitative Correlation between ATPase Activity and Vectorial Copper Transport

Journal of the American Chemical Society, 2017
Cu-ATPases are membrane copper transporters present in all kingdoms of life. They play a central role in Cu homeostasis by pumping Cu ions across cell membranes with energy derived from ATP hydrolysis. In this work, the Cu-ATPase CopA from Escherichia coli was expressed and purified in fully functional form and demonstrated to bind Cu(I) with ...
Chathuri J. K. Wijekoon, Saumya R. Udagedara, Roland L. Knorr, Rumiana Dimova, Anthony G. Wedd, Zhiguang Xiao   +5 more
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