Function and Regulation of the Mammalian Copper-transporting ATPases: Insights from Biochemical and Cell Biological Approaches

The Wilson disease gene is a putative copper transporting P–type ATPase similar to the Menkes gene

1993 • 1,905 citations

Isolation of a candidate gene for Menkes disease and evidence that it encodes a copper–transporting ATPase

1993 • 1,395 citations

The Wilson disease gene is a copper transporting ATPase with homology to the Menkes disease gene

1993 • 1,345 citations

Isolation of a candidate gene for Menkes disease that encodes a potential heavy metal binding protein

1993 • 720 citations

Isolation of a partial candidate gene for Menkes disease by positional cloning

1993 • 690 citations

Ligand-regulated transport of the Menkes copper P-type ATPase efflux pump from the Golgi apparatus to the plasma membrane: a novel mechanism of regulated trafficking.

1996 • 623 citations

Menkes's kinky hair syndrome. An inherited defect in copper absorption with widespread effects.

1972 • 550 citations

Isolation and Characterization of a Human Liver cDNA as a Candidate Gene for Wilson Disease

1993 • 501 citations

CPx-type ATPases: a class of P-type ATPases that pump heavy metals

1996 • 472 citations

Organization of P-type ATPases: significance of structural diversity

1995 • 448 citations

The Menkes/Wilson disease gene homologue in yeast provides copper to a ceruloplasmin-like oxidase required for iron uptake.

1995 • 431 citations

The LEC rat has a deletion in the copper transporting ATPase gene homologous to the Wilson disease gene

1994 • 382 citations

Characterization of the Wilson disease gene encoding a P-type copper transporting ATPase: genomic organization, alternative splicing, and structure/function predictions

1994 • 355 citations

Biochemical Characterization of the Wilson Disease Protein and Functional Expression in the Yeast Saccharomyces cerevisiae

1997 • 324 citations

Protein targeting by tyrosine- and di-leucine-based signals: evidence for distinct saturable components.

1996 • 314 citations

Genetic and molecular basis for copper toxicity

1996 • 287 citations

Interaction of the copper chaperone HAH1 with the Wilson disease protein is essential for copper homeostasis

1999 • 267 citations

Copper-induced apical trafficking of ATP7B in polarized hepatoma cells provides a mechanism for biliary copper excretion

2000 • 260 citations

Evolution of P-type ATPases

1998 • 241 citations

N-terminal Domains of Human Copper-transporting Adenosine Triphosphatases (the Wilson's and Menkes Disease Proteins) Bind Copper Selectively in Vivo and in Vitro with Stoichiometry of One Copper Per Metal-binding Repeat

1997 • 237 citations

Functional expression of the Wilson disease protein reveals mislocalization and impaired copper-dependent trafficking of the common H1069Q mutation

1998 • 225 citations

The metallochaperone Atox1 plays a critical role in perinatal copper homeostasis

2001 • 220 citations

The Menkes copper transporter is required for the activation of tyrosinase

2000 • 213 citations

X-Linked Cutis Laxa

1980 • 206 citations

Biochemical characterization and intracellular localization of the Menkes disease protein

1996 • 205 citations

Disorders of copper transport

1995 • 195 citations

Evidence for an Aspartyl Phosphate Residue at the Active Site of Sodium and Potassium Ion Transport Adenosine Triphosphatase

1973 • 183 citations

Menkes disease: a biochemical abnormality in cultured human fibroblasts.

1976 • 172 citations

The Role of GMXCXXC Metal Binding Sites in the Copper-induced Redistribution of the Menkes Protein

1999 • 167 citations

Restoration of Holoceruloplasmin Synthesis in LEC Rat after Infusion of Recombinant Adenovirus Bearing WND cDNA

1998 • 167 citations

Copper-dependent trafficking of Wilson disease mutant ATP7B proteins

2000 • 167 citations

Role of the Copper-binding Domain in the Copper Transport Function of ATP7B, the P-type ATPase Defective in Wilson Disease

1999 • 163 citations

Wilson's Disease

2000 • 161 citations

Expression, Purification, and Metal Binding Properties of the N-terminal Domain from the Wilson Disease Putative Copper-transporting ATPase (ATP7B)

1997 • 160 citations

Functional Expression of the Menkes Disease Protein Reveals Common Biochemical Mechanisms Among the Copper-transporting P-type ATPases

1998 • 158 citations

The Lys1010–Lys1325 Fragment of the Wilson's Disease Protein Binds Nucleotides and Interacts with the N-terminal Domain of This Protein in a Copper-dependent Manner

2001 • 154 citations

The Wilson's disease gene and phenotypic diversity

2001 • 146 citations

COPPER INCORPORATION STUDIES ON CULTURED CELLS FOR PRENATAL DIAGNOSIS OF MENKES' DISEASE

1976 • 128 citations

The Regulation of Catalytic Activity of the Menkes Copper-translocating P-type ATPase

2001 • 126 citations

Diagnosis and therapy of Menkes syndrome, a genetic form of copper deficiency

1998 • 125 citations

Functional consequences of alterations to amino acids located in the nucleotide binding domain of the Ca2(+)-ATPase of sarcoplasmic reticulum.

1990 • 115 citations

Correction of the Copper Transport Defect of Menkes Patient Fibroblasts by Expression of the Menkes and Wilson ATPases

1998 • 114 citations

Copper Specifically Regulates Intracellular Phosphorylation of the Wilson's Disease Protein, a Human Copper-transporting ATPase

2001 • 111 citations

Copper-Induced Conformational Changes in the N-Terminal Domain of the Wilson Disease Copper-Transporting ATPase

2000 • 109 citations

Abnormal copper metabolism and deficient lysyl oxidase activity in a heritable connective tissue disorder.

1982 • 108 citations

Functional Properties of the Copper-transporting ATPase ATP7B (The Wilson's Disease Protein) Expressed in Insect Cells

2002 • 105 citations

Localization of the Wilson's disease protein in human liver

1999 • 103 citations

Analysis of functional domains of Wilson disease protein (ATP7B) in Saccharomyces cerevisiae

1998 • 100 citations

Developmental expression of the mouse mottled and toxic milk genes suggests distinct functions for the Menkes and Wilson disease copper transporters

1997 • 93 citations

Mutation Analysis and Expression of the Mottled Gene in the Macular Mouse Model of Menkes Disease

1997 • 88 citations

Identification of a di-leucine motif within the C terminus domain of the Menkes disease protein that mediates endocytosis from the plasma membrane

1999 • 84 citations

IV. Wilson’s disease and Menkes disease

1999 • 81 citations

Characterization of the Menkes Protein Copper-Binding Domains and Their Role in Copper-Induced Protein Relocalization

1999 • 77 citations

Functional Studies on the Wilson Copper P-Type ATPase and Toxic Milk Mouse Mutant

2001 • 77 citations

ATP‐dependent copper transport by the Menkes protein in membrane vesicles isolated from cultured Chinese hamster ovary cells

1998 • 75 citations

Intracellular distribution of the Wilson's disease gene product (atpase7b) afterin vitro andin vivo exogenous expression in hepatocytes from the LEC rat, an animal model of Wilson's disease

1998 • 71 citations

Distinctive Menkes disease variant with occipital horns: Delineation of natural history and clinical phenotype

1996 • 63 citations

Expression of the Menkes gene homologue in mouse tissues lack of effect of copper on the mRNA levels

1994 • 62 citations

The Menkes Disease Protein Binds Copper via Novel 2-Coordinate Cu(I)−Cysteinates in the N-Terminal Domain

1998 • 59 citations

Disorder of copper transport

2001 • 58 citations

Structure and function of the P-type ATPases

1999 • 52 citations

Stoichiometry of Complex Formation between Copper(I) and the N-Terminal Domain of the Menkes Protein

2000 • 50 citations

Interaction of Nucleotides with Asp351 and the Conserved Phosphorylation Loop of Sarcoplasmic Reticulum Ca2+-ATPase

1999 • 43 citations

Importance of Thr-353 of the Conserved Phosphorylation Loop of the Sarcoplasmic Reticulum Ca2+-ATPase in MgATP Binding and Catalytic Activity

2001 • 42 citations

Immunohistochemical determination of the Wilson Copper-transporting P-type ATPase in the brain tissues of the rat

1999 • 41 citations

Cooperative binding of copper(I) to the metal binding domains in Menkes disease protein

1999 • 40 citations

Biliary copper excretion capacity in intact animals: Correlation between ATP7B function, hepatic mass, and biliary copper excretion

2000 • 33 citations

Impaired Hepatic Copper Homeostasis in Long-Evans Cinnamon Rats: Reduced Biliary Excretion of Copper

1994 • 27 citations

Role of conserved TGDGVND-loop in Mg2+ binding, phosphorylation, and energy transfer in Na,K-ATPase.

2001 • 27 citations

Characterisation of copper-binding to the second sub-domain of the Menkes protein ATPase (MNKr2)

1999 • 23 citations

Expression, purification and copper‐binding studies of the first metal‐binding domain of Menkes protein

1999 • 21 citations

The Wilson's disease protein expressed in Sf9 cells is phosphorylated

2002 • 17 citations

Biliary Excretion of Copper, Metallothionein, and Glutathione into Long-Evans Cinnamon Rats: A Convincing Animal Model for Wilson Disease

1995 • 17 citations

Abnormalities of copper accumulation in cell lines established from nine different alleles of mottled are the same as those found in Menkes disease.

1997 • 14 citations

[Mild form of Menkes syndrome].

1984 • 7 citations