• Publications
  • Influence
Copper inhibits beta-amyloid production and stimulates the non-amyloidogenic pathway of amyloid-precursor-protein secretion.
Previous studies have demonstrated that amyloid precursor protein (APP) can bind and reduce Cu(II) to Cu(I), leading to oxidative modification of APP. In the present study we show that adding copperExpand
  • 142
  • 11
Copper-regulated Trafficking of the Menkes Disease Copper ATPase Is Associated with Formation of a Phosphorylated Catalytic Intermediate*
The Menkes protein (MNK; ATP7A) is a copper-transporting P-type ATPase that is defective in the copper deficiency disorder, Menkes disease. MNK is localized in thetrans-Golgi network and transportsExpand
  • 125
  • 10
The Regulation of Catalytic Activity of the Menkes Copper-translocating P-type ATPase
The Menkes protein is a transmembrane copper translocating P-type ATPase. Mutations in the Menkes gene that affect the function of the Menkes protein may cause Menkes disease in humans, which isExpand
  • 118
  • 8
Essential roles in development and pigmentation for the Drosophila copper transporter DmATP7.
Defects in the mammalian Menkes and Wilson copper transporting P-type ATPases cause severe copper homeostasis disease phenotypes in humans. Here, we find that DmATP7, the sole Drosophila orthologueExpand
  • 68
  • 7
Copper homoeostasis in Drosophila melanogaster S2 cells.
Copper homoeostasis was investigated in the Drosophila melanogaster S2 cell line to develop an insect model for the study of copper regulation. Real-time PCR studies have demonstrated expression inExpand
  • 60
  • 7
Copper levels are increased in the cerebral cortex and liver of APP and APLP2 knockout mice
The pathological process in Alzheimer's disease (AD) involves amyloid beta (Abeta) deposition and neuronal cell degeneration. The neurotoxic Abeta peptide is derived from the amyloid precursorExpand
  • 259
  • 6
Functional Analysis of the N-terminal CXXC Metal-binding Motifs in the Human Menkes Copper-transporting P-type ATPase Expressed in Cultured Mammalian Cells*
The Menkes protein (MNK) is a copper-transporting P-type ATPase, which has six highly conserved metal-binding sites, GMTCXXC, at the N terminus. The metal-binding sites may be involved in MNKExpand
  • 131
  • 6
Direct evidence that mitochondrial iron accumulation occurs in Friedreich ataxia
Friedreich ataxia (FRDA) is due to mutations in the FRDA gene (FRDA). When the gene homologous to FRDA is knocked out in yeast, there is accumulation of iron in mitochondria and reduced respiratoryExpand
  • 150
  • 5
Purification and membrane reconstitution of catalytically active Menkes copper-transporting P-type ATPase (MNK; ATP7A).
The MNK (Menkes disease protein; ATP7A) is a major copper- transporting P-type ATPase involved in the delivery of copper to cuproenzymes in the secretory pathway and the efflux of excess copper fromExpand
  • 41
  • 5
Expression and localisation of the essential copper transporter DmATP7 in Drosophila neuronal and intestinal tissues.
Copper homeostasis is achieved by a combination of regulated uptake, efflux and sequestration and is essential for animal health and viability. Transmembrane copper transport proteins of the P-typeExpand
  • 40
  • 5