| Title |
Human Copper-Transporting ATPase ATP7B (The Wilson's Disease Protein): Biochemical Properties and Regulation
|
|---|---|
| Published in |
Journal of Bioenergetics and Biomembranes, October 2002
|
| DOI | 10.1023/a:1021297919034 |
| Pubmed ID | |
| Authors |
Svetlana Lutsenko, Roman G. Efremov, Ruslan Tsivkovskii, Joel M. Walker |
| Abstract |
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. Copper regulates WNDP at several levels, modulating its catalytic activity, posttranslational modification, and intracellular localization. This review summarizes recent studies on enzymatic function and copper-dependent regulation of WNDP. Specifically, we describe the molecular architecture and major biochemical properties of WNDP, discuss advantages of the recently developed functional expression of WNDP in insect cells, and summarize the results of the ligand-binding studies and molecular modeling experiments for the ATP-binding domain of WNDP. In addition, we speculate on how copper binding may regulate the activity and intracellular distribution of WNDP, and what role the human copper chaperone Atox1 may play in these processes. |
Mendeley readers
The data shown below were compiled from readership statistics for 58 Mendeley readers of this research output. Click here to see the associated Mendeley record.
Geographical breakdown
| Country | Count | As % |
|---|---|---|
| Unknown | 58 | 100% |
Demographic breakdown
| Readers by professional status | Count | As % |
|---|---|---|
| Student > Ph. D. Student | 13 | 22% |
| Researcher | 8 | 14% |
| Student > Master | 6 | 10% |
| Student > Bachelor | 6 | 10% |
| Professor > Associate Professor | 5 | 9% |
| Other | 15 | 26% |
| Unknown | 5 | 9% |
| Readers by discipline | Count | As % |
|---|---|---|
| Biochemistry, Genetics and Molecular Biology | 19 | 33% |
| Agricultural and Biological Sciences | 12 | 21% |
| Medicine and Dentistry | 5 | 9% |
| Chemistry | 3 | 5% |
| Neuroscience | 3 | 5% |
| Other | 5 | 9% |
| Unknown | 11 | 19% |
Attention Score in Context
This research output has an Altmetric Attention Score of 3. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 04 August 2015.
All research outputs
#8,534,528
of 25,373,627 outputs
Outputs from Journal of Bioenergetics and Biomembranes
#115
of 491 outputs
Outputs of similar age
#17,268
of 49,678 outputs
Outputs of similar age from Journal of Bioenergetics and Biomembranes
#1
of 4 outputs
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So far Altmetric has tracked 491 research outputs from this source. They receive a mean Attention Score of 3.4. This one is in the 39th percentile – i.e., 39% of its peers scored the same or lower than it.
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