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Animal models of Wilson disease

Journal of neurochemistry, 2018-08, Vol.146 (4), p.356-373

2018

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Human copper transporter ATP7B (Wilson disease protein) forms stable dimers in vitro and in cells

The Journal of biological chemistry, 2017-11, Vol.292 (46), p.18760-18774

2017

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The metal chaperone Atox1 regulates the activity of the human copper transporter ATP7B by modulating domain dynamics

The Journal of biological chemistry, 2017-11, Vol.292 (44), p.18169-18177

2017

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Metallothioneins regulate ATP7A trafficking and control cell viability during copper deficiency and excess

Scientific reports, 2020-05, Vol.10 (1), p.7856, Article 7856

2020

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Copper ions are novel therapeutic agents for uterine leiomyosarcoma

American journal of obstetrics and gynecology, 2020-01, Vol.222 (1), p.64.e1-64.e16

2020

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Tetrathiomolybdate induces dimerization of the metal-binding domain of ATPase and inhibits platination of the protein

Nature communications, 2019-01, Vol.10 (1), p.186-186, Article 186

2019

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Targeted inactivation of copper transporter Atp7b in hepatocytes causes liver steatosis and obesity in mice

American journal of physiology: Gastrointestinal and liver physiology, 2017-07, Vol.313 (1), p.G39-G49

2017

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Copper resistance in the cold: Genome analysis and characterisation of a P IB-1 ATPase in Bizionia argentinensis

Environmental microbiology reports, 2024-08, Vol.16 (4), p.e13278

2024

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Expression and copper binding properties of the N-terminal domain of copper P-type ATPases of African trypanosomes

Molecular and biochemical parasitology, 2020-01, Vol.235, p.111245, Article 111245

2020

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An MTF1 binding site disrupted by a homozygous variant in the promoter of ATP7B likely causes Wilson Disease

European journal of human genetics : EJHG, 2018-12, Vol.26 (12), p.1810-1818

2018

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Akt2 (Protein Kinase B Beta) Stabilizes ATP7A, a Copper Transporter for Extracellular Superoxide Dismutase, in Vascular Smooth Muscle: Novel Mechanism to Limit Endothelial Dysfunction in Type 2 Diabetes Mellitus

Arteriosclerosis, thrombosis, and vascular biology, 2018-03, Vol.38 (3), p.529-541

2018

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Identification of a copper-transporting ATPase involved in biosynthesis of A. flavus conidial pigment

Applied microbiology and biotechnology, 2019-06, Vol.103 (12), p.4889-4897

2019

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Direct Interaction of ATP7B and LC3B Proteins Suggests a Cooperative Role of Copper Transportation and Autophagy

Cells (Basel, Switzerland), 2021-11, Vol.10 (11), p.3118

2021

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Ammonium tetrathiomolybdate treatment targets the copper transporter ATP7A and enhances sensitivity of breast cancer to cisplatin

Oncotarget, 2016-12, Vol.7 (51), p.84439-84452

2016

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Genetic analysis of ATP7B in 102 south Indian families with Wilson disease

PloS one, 2019-05, Vol.14 (5), p.e0215779-e0215779

2019

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The Structure of Metal Binding Domain 1 of the Copper Transporter ATP7B Reveals Mechanism of a Singular Wilson Disease Mutation

Scientific reports, 2018-01, Vol.8 (1), p.581-6, Article 581

2018

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Homozygous frame shift variant in ATP7B exon 1 leads to bypass of nonsense-mediated mRNA decay and to a protein capable of copper export

European journal of human genetics : EJHG, 2019-06, Vol.27 (6), p.879-887

2019

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The six metal binding domains in human copper transporter, ATP7B: molecular biophysics and disease-causing mutations

Biometals, 2017-12, Vol.30 (6), p.823-840

2017

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Unraveling the Impact of Cysteine-to-Serine Mutations on the Structural and Functional Properties of Cu(I)-Binding Proteins

International journal of molecular sciences, 2019-07, Vol.20 (14), p.3462

2019

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Association of the canine ATP7A and ATP7B with hepatic copper accumulation in Dobermann dogs

Journal of veterinary internal medicine, 2019-07, Vol.33 (4), p.1646-1652

2019

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A glimpse into the regulation of the Wilson disease protein, ATP7B, sheds light on the complexity of mammalian apical trafficking pathways

Metallomics, 2018-03, Vol.10 (3), p.378-387

2018

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In-silico analysis of novel p.(Gly14Ser) variant of ATOX1 gene: plausible role in modulating ATOX1–ATP7B interaction

Molecular biology reports, 2019-06, Vol.46 (3), p.3307-3313

2019

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Regulation of the apico-basolateral trafficking polarity of the homologous copper-ATPases ATP7A and ATP7B

Journal of cell science, 2024-03, Vol.137 (5)

2024

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Single-cell tracking demonstrates copper chaperone Atox1 to be required for breast cancer cell migration

Proceedings of the National Academy of Sciences - PNAS, 2020-01, Vol.117 (4), p.2014-2019

2020

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Ceruloplasmin gene expression profile changes in the rat mammary gland during pregnancy, lactation and involution

Journal of trace elements in medicine and biology, 2017-09, Vol.43, p.126-134

2017

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Animal models of Wilson disease

Human copper transporter ATP7B (Wilson disease protein) forms stable dimers in vitro and in cells

The metal chaperone Atox1 regulates the activity of the human copper transporter ATP7B by modulating domain dynamics

Metallothioneins regulate ATP7A trafficking and control cell viability during copper deficiency and excess

Copper ions are novel therapeutic agents for uterine leiomyosarcoma

Tetrathiomolybdate induces dimerization of the metal-binding domain of ATPase and inhibits platination of the protein

Targeted inactivation of copper transporter Atp7b in hepatocytes causes liver steatosis and obesity in mice

Copper resistance in the cold: Genome analysis and characterisation of a P IB-1 ATPase in Bizionia argentinensis

Expression and copper binding properties of the N-terminal domain of copper P-type ATPases of African trypanosomes

An MTF1 binding site disrupted by a homozygous variant in the promoter of ATP7B likely causes Wilson Disease

Akt2 (Protein Kinase B Beta) Stabilizes ATP7A, a Copper Transporter for Extracellular Superoxide Dismutase, in Vascular Smooth Muscle: Novel Mechanism to Limit Endothelial Dysfunction in Type 2 Diabetes Mellitus

Identification of a copper-transporting ATPase involved in biosynthesis of A. flavus conidial pigment

Direct Interaction of ATP7B and LC3B Proteins Suggests a Cooperative Role of Copper Transportation and Autophagy

Ammonium tetrathiomolybdate treatment targets the copper transporter ATP7A and enhances sensitivity of breast cancer to cisplatin

Genetic analysis of ATP7B in 102 south Indian families with Wilson disease

The Structure of Metal Binding Domain 1 of the Copper Transporter ATP7B Reveals Mechanism of a Singular Wilson Disease Mutation

Homozygous frame shift variant in ATP7B exon 1 leads to bypass of nonsense-mediated mRNA decay and to a protein capable of copper export

The six metal binding domains in human copper transporter, ATP7B: molecular biophysics and disease-causing mutations

Unraveling the Impact of Cysteine-to-Serine Mutations on the Structural and Functional Properties of Cu(I)-Binding Proteins

Association of the canine ATP7A and ATP7B with hepatic copper accumulation in Dobermann dogs

A glimpse into the regulation of the Wilson disease protein, ATP7B, sheds light on the complexity of mammalian apical trafficking pathways

In-silico analysis of novel p.(Gly14Ser) variant of ATOX1 gene: plausible role in modulating ATOX1–ATP7B interaction

Regulation of the apico-basolateral trafficking polarity of the homologous copper-ATPases ATP7A and ATP7B

Single-cell tracking demonstrates copper chaperone Atox1 to be required for breast cancer cell migration

Ceruloplasmin gene expression profile changes in the rat mammary gland during pregnancy, lactation and involution

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