| Atp7b-dependent choroid plexus dysfunction causes transient copper deficit and metabolic changes in the developing mouse brain. | Atp7b-dependent choroid plexus dysfunction causes transient copper deficit and metabolic changes in the developing mouse brain.
Washington-Hughes CL, Roy S, Seneviratne HK, Karuppagounder SS, Morel Y, Jones JW, Zak A, Xiao T, Boronina TN, Cole RN, Bumpus NN, Chang CJ, Dawson TM, Lutsenko S., Free PMC Article | 02/4/2023 |
| Systemic deletion of Atp7b modifies the hepatocytes' response to copper overload in the mouse models of Wilson disease. | Systemic deletion of Atp7b modifies the hepatocytes' response to copper overload in the mouse models of Wilson disease.
Muchenditsi A, Talbot CC Jr, Gottlieb A, Yang H, Kang B, Boronina T, Cole R, Wang L, Dev S, Hamilton JP, Lutsenko S., Free PMC Article | 12/11/2021 |
| Copper Sulfide Facilitates Hepatobiliary Clearance of Gold Nanoparticles through the Copper-Transporting ATPase ATP7B. | Copper Sulfide Facilitates Hepatobiliary Clearance of Gold Nanoparticles through the Copper-Transporting ATPase ATP7B.
Wang X, Guo L, Zhang S, Chen Y, Chen YT, Zheng B, Sun J, Qian Y, Chen Y, Yan B, Lu W., Free PMC Article | 08/22/2020 |
| Atp7b (-/-) mice showed blunted body-weight gain over time, had lower fat mass, and were more glucose tolerant than wild type (WT) littermate controls. | Metabolic dysregulation in the Atp7b(-/-) Wilson's disease mouse model.
Wooton-Kee CR, Robertson M, Zhou Y, Dong B, Sun Z, Kim KH, Liu H, Xu Y, Putluri N, Saha P, Coarfa C, Moore DD, Nuotio-Antar AM., Free PMC Article | 06/6/2020 |
| our study demonstrated that adeno-associated virus gene therapy based on truncated ATP7B is a promising strategy in the treatment of Wilson's disease | Long-Term Correction of Copper Metabolism in Wilson's Disease Mice with AAV8 Vector Delivering Truncated ATP7B.
Leng Y, Li P, Zhou L, Xiao L, Liu Y, Zheng Z, Qin F, Hao Q, Xu H, Yao S, Dong B. | 06/6/2020 |
| Duodenal iron content was also lower in Atp7b((-/-)) mice, but did not reach statistical significance. The results of our study suggest that metallothionein is elevated in the liver and duodenum of Atp7b((-/-)) mice. | Metallothionein is elevated in liver and duodenum of Atp7b((-/-)) mice.
Zhang CC, Volkmann M, Tuma S, Stremmel W, Merle U. | 06/1/2019 |
| the copper transporter ATP7A is necessary for the activity of LOX and LOXL enzymes. Silencing of ATP7A inhibited LOX activity in the 4T1 mammary carcinoma cell line. | ATP7A delivers copper to the lysyl oxidase family of enzymes and promotes tumorigenesis and metastasis.
Shanbhag V, Jasmer-McDonald K, Zhu S, Martin AL, Gudekar N, Khan A, Ladomersky E, Singh K, Weisman GA, Petris MJ., Free PMC Article | 05/25/2019 |
| DBH-containing neurons express both ATP7A and ATP7B. The two transporters are located in distinct cellular compartments and oppositely regulate the export of soluble DBH from cultured neuronal cells under resting conditions. | ATP7A and ATP7B copper transporters have distinct functions in the regulation of neuronal dopamine-β-hydroxylase.
Schmidt K, Ralle M, Schaffer T, Jayakanthan S, Bari B, Muchenditsi A, Lutsenko S., Free PMC Article | 04/13/2019 |
| Copper homeostasis in the testes is closely controlled by copper-transporting ATPases ATP7A and ATP7B proteins. | Atp7a and Atp7b regulate copper homeostasis in developing male germ cells in mice.
Ogórek M, Lenartowicz M, Starzyński R, Jończy A, Staroń R, Doniec A, Krzeptowski W, Bednarz A, Pierzchała O, Lipiński P, Rajfur Z, Baster Z, Gibas-Tybur P, Grzmil P. | 05/26/2018 |
| This study showed that knockout of Atp7b led to altered lipid metabolism and liver steatosis in the absence of inflammation. | Targeted inactivation of copper transporter Atp7b in hepatocytes causes liver steatosis and obesity in mice.
Muchenditsi A, Yang H, Hamilton JP, Koganti L, Housseau F, Aronov L, Fan H, Pierson H, Bhattacharjee A, Murphy R, Sears C, Potter J, Wooton-Kee CR, Lutsenko S., Free PMC Article | 08/19/2017 |
| ATP7B is transported from the trans-Golgi network (TGN) to the bile canaliculus by basolateral sorting and endocytosis, and microtubule-mediated transcytosis through the subapical compartment. | Basolateral sorting and transcytosis define the Cu+-regulated translocation of ATP7B to the bile canaliculus.
Lalioti V, Peiró R, Pérez-Berlanga M, Tsuchiya Y, Muñoz A, Villalba T, Sanchez C, Sandoval IV. | 08/12/2017 |
| Data indicate that the copper-transporting ATPase gene (Atp7b) knockout mice showed a drastic, time-dependent accumulation of hepatic copper. | Laser ablation inductively coupled plasma mass spectrometry imaging of metals in experimental and clinical Wilson's disease.
Boaru SG, Merle U, Uerlings R, Zimmermann A, Flechtenmacher C, Willheim C, Eder E, Ferenci P, Stremmel W, Weiskirchen R., Free PMC Article | 04/2/2016 |
| The aim of this study was to identify copper disturbances according to various brain compartments and further dissect the causal relationship between copper storage and neuronal damage using Atp7b(-/-) mice. | The discrepancy between the absence of copper deposition and the presence of neuronal damage in the brain of Atp7b(-/-) mice.
Dong Y, Shi SS, Chen S, Ni W, Zhu M, Wu ZY. | 10/31/2015 |
| DKWSLLL sequence is essential for ATP7b sorting at the TGN, transport from the TGN to the PM, endocytosis, and recycling to the TGN and PM. | DKWSLLL, a versatile DXXXLL-type signal with distinct roles in the Cu(+)-regulated trafficking of ATP7B.
Lalioti V, Hernandez-Tiedra S, Sandoval IV. | 04/11/2015 |
| Ligand-activated nuclear receptors FXR/NR1H4 and GR/NR3C1 and nuclear receptor interacting partners are less abundant in Atp7b(-/-) hepatocyte nuclei. | A systems approach implicates nuclear receptor targeting in the Atp7b(-/-) mouse model of Wilson's disease.
Wilmarth PA, Short KK, Fiehn O, Lutsenko S, David LL, Burkhead JL., Free PMC Article | 11/3/2012 |
| By performing dynamic PET, authors obtained the first real-time measurements of 64Cu distribution in the organs or tissues of Atp7b -/- mice. | Positron emission tomography of copper metabolism in the Atp7b-/- knock-out mouse model of Wilson's disease.
Peng F, Lutsenko S, Sun X, Muzik O., Free PMC Article | 08/11/2012 |
| Clusterin and COMMD1 independently regulate degradation of the mammalian copper ATPases ATP7A and ATP7B. | Clusterin and COMMD1 independently regulate degradation of the mammalian copper ATPases ATP7A and ATP7B.
Materia S, Cater MA, Klomp LW, Mercer JF, La Fontaine S., Free PMC Article | 03/17/2012 |
| Neuroinflammatory and behavioural changes in the Atp7B mutant mouse model of Wilson's disease. | Neuroinflammatory and behavioural changes in the Atp7B mutant mouse model of Wilson's disease.
Terwel D, Löschmann YN, Schmidt HH, Schöler HR, Cantz T, Heneka MT. | 08/13/2011 |
| cisplatin binding to ATP7B and/or general changes in cellular copper homeostasis are likely contributors to the increased resistance to the drug. | Functional interactions of Cu-ATPase ATP7B with cisplatin and the role of ATP7B in the resistance of cells to the drug.
Leonhardt K, Gebhardt R, Mössner J, Lutsenko S, Huster D., Free PMC Article | 01/21/2010 |
| Studies using polarized PMC42-LA cells that overexpressed mAtp7B protein showed that this transporter facilitates copper efflux from the apical surface of the cells. | ATP7B expression in human breast epithelial cells is mediated by lactational hormones.
Michalczyk A, Bastow E, Greenough M, Camakaris J, Freestone D, Taylor P, Linder M, Mercer J, Ackland ML., Free PMC Article | 01/21/2010 |
| review of quantitative detection of copper-transporting ATPases in tissues and distinct biochemical characteristics and dissimilar trafficking properties of ATP7A and ATP7B in cells, in which they are co-expressed, indicating distinct specific functions | Copper-transporting ATPases ATP7A and ATP7B: cousins, not twins.
Linz R, Lutsenko S. | 01/21/2010 |
| Both Cu-ATPases (ATP7A and ATP7b) regulate renal copper, with ATP7A playing a major role in exporting copper via basolateral membranes and protecting renal tissue against copper overload. | Intracellular targeting of copper-transporting ATPase ATP7A in a normal and Atp7b-/- kidney.
Linz R, Barnes NL, Zimnicka AM, Kaplan JH, Eipper B, Lutsenko S. | 01/21/2010 |
| Both Atp7a and Atp7b are expressed in glomeruli; however, Atp7b is also seen in the kidney medulla suggesting that glomeruli are responsible for regulating copper levels in the filtrate | Expression in mouse kidney of membrane copper transporters Atp7a and Atp7b.
Moore SD, Cox DW. | 01/21/2010 |
| in Atp7b-/- mice elevated copper affects specific cellular targets at the transcription and/or translation levels and has distinct effects on liver metabolic function, prior to appearance of histopathological changes | High copper selectively alters lipid metabolism and cell cycle machinery in the mouse model of Wilson disease.
Huster D, Purnat TD, Burkhead JL, Ralle M, Fiehn O, Stuckert F, Olson NE, Teupser D, Lutsenko S. | 01/21/2010 |
| In mice in which this enzyme has been knocked out, the is a reduced level of beta-hydroxylase and norepinephrine in the adrenal gland. | A mutation in the ATP7B copper transporter causes reduced dopamine beta-hydroxylase and norepinephrine in mouse adrenal.
Gerbasi V, Lutsenko S, Lewis EJ. | 01/21/2010 |