Machine generated contents note: I. COPPER IN MAMMALS -- 1 Biochemistry and Molecular Biology of Copper in Mammals 3 -- Maria C. Linder -- II. COPPER PROTEINS -- 2 Biochemistry of the Wilson's Disease Protein 53 -- Svetlana Lutsenko, Ruslan Tsivkovskii, Matthew J. Cooper, -- Brian C. MacArthur, and Hans-Peter Bichinger -- 3 Biologically Relevant Properties of Copper-Containing Proteins -- Analyzed by Means of Semiquantitative and Quantitative -- Theoretical Descriptors 35 -- Francesca De Rienzo, R. Razif Gabdoulline, Rebecca C. Wade, -- and M. Cristina Menziani -- 4 Molecular Modeling and Dynamics of Copper Proteins 18 -- Mattia Falconi and Alessandro Desideri -- 5 The Prion Protein and Copper: What Is the Connection ?. 301 -- Lesley R. Brown and David A. Harris -- 6 Prion Protein: A Synaptic Cuproprotein. 511 -- David R. Brown -- 7 Cytochrome-c Oxidase 131 -- Shinya Yoshikawa -- 8 The Fet3 Protein: A Multicopper Ferroxidase Essential to Iron -- M etabolism in Yeast 351 -- Daniel J. Kosman -- 9 Interaction of Copper-Binding Proteins from Enterococcus hirae. 771 -- Paul A. Cobine, Christopher E. Jones, -- Wasantha A. Wickramasinghe, Marc Solioz, -- and Charles T. Dameron -- III. COPPER TRANSPORT -- 01 Copper Uptake in Eukaryotic Cells 981 -- Lisbeth Birk Moller and Nina Horn -- 11 The Role of PINA in Copper Transport, Circadian Rhythms, -- and Wilson's Disease 102 -- Jimo Borjigin, Xing Sun, and Michael M. Wang -- 21 The Identification of Motifs Involved in the Intracellular -- Trafficking of the Menkes Disease Protein 902 -- Michael J. Francis and Anthony P. Monaco -- 31 Intracellular Copper Transport and ATP7B, the Wilson's -- Disease Protein 722 -- Kunihiko Terada and Toshihiro Sugiyama -- 41 A Multicopper Oxidase-Based Iron-Transport System -- in Yeast 532 -- Jerry Kaplan -- IV. MOLECULAR PATHOGENESIS OF DISEASES OF COPPER METABOLISM -- 51 Molecular Basis of Diseases of Copper Homeostasis 942 -- Julian F. B. Mercer, David Kramer, and James Camakaris -- 61 Disturbances of Copper Homeostasis and Brain Function 772 -- Giuseppe Rotilio, Maria Rosa Ciriolo, Maria Teresa Cari, -- and Luisa Rossi -- 71 Role of Copper and Other Transition Metal Ions in the -- Pathogenesis of Parkinson's Disease, Prion Diseases, -- Familial Amytrophic Lateral Sclerosis -- and Alzheimer's Disease 792 -- Gerd Multhaup, Hermann H. Dieter, Konrad Beyreuther, -- and Thomas A. Bayer -- 81 Molecular Genetics and Clinical Aspects of Menkes Disease -- and Occipital Horn Syndrome 913 -- Hiroko Kodama -- 91 The Meaning of Alternative Transcripts of the Menkes -- Disease Gene 333 -- EdwardD. Harris, Manchi C. M. Reddy, and Sudeep Majumdar -- 02 Expression Profiling in Menkes Disease 343 -- Zeynep Tiimer, Nina Horn, and Lars Riff Jensen -- 12 Regulation by Copper of the Expression of Human Bis, a New -- Gene Involved in the Cellular Stress Response to Metals 753 -- Maria Gabriella Pagliuca, Rosa Lerose, and Arturo Leone -- 22 Non-Indian Childhood Cirrhosis: Using a Founder Population -- to Identify the Underlying Genetic Defect 963 -- Cisca Wijmenga -- V. COPPER TOXICITY AND THERAPEUTICS -- 32 Biological Effects of Chronic Copper Exposure 583 -- Magdalena Araya, Mauricio Gonzdlez, Manuel Olivares, -- and Ricardo Uauy -- 42 Copper Toxicity to Tight Junctions in the Human Intestinal -- Caco-2 Cell Line 793 -- Simonetta Ferruzza, Yula Sambuy, Andrea Onetti-Muda, -- Fabio Nobili, and Maria Laura Scarino -- 52 Cellular Responses to Copper in Aquatic Organisms: Importance -- of Oxidative Stress and Alteration of Signal Transduction 714 -- Aldo Viarengo, Bruno Burlando, and Claudia Bolognesi -- 62 Selective Removal of Copper Accumulating in the Form Bound -- to Metallothionein by Tetrathiomolybdate 334 -- Kazuo T. Suzuki and Yasumitsu Ogra -- 72 Control of Copper in Wilson's Disease and Diseases -- of Neovascularization, such as Cancer 344 -- George J. Brewer -- 82 Copper-Rich Metallothionein Polymers During -- the Development of Fulminant Hepatitis in LEC Rats: -- Effect of D-Penicillamine 164 -- Dominik Klein, Josef Lichtmannegger, and Karl H. Summer -- VI. COPPER METABOLISM AND HOMEOSTASIS -- 92 Sensory Protein Modification: A New Feature -- in Copper Balancing 574 -- Zhiwu Zhu, Jose Heredia, and Ryan Teves -- 03 Comparative Analysis of Copper and Iron Metabolism -- in Photosynthetic Eukaryotes vs Yeast and Mammals 184 -- Sharon La Fontaine, Jeanette Quinn, and Sabeeha Merchant -- 13 Copper and the Morphological Development of Streptomyces 305 -- Erik Vijgenboom and Bart Keijser -- 23 Molecular Hardware of Copper Homeostasis -- in Enterococcus hirae 725 -- Reinhard Wimmer, Charles T. Dameron, and Marc Solioz -- 33 Copper Homeostasis in Plants 345 -- Helena Mira, Vicente Sancendn, and Lola Penarrubia -- 43 The Role of Copper Ions in Regulating Methane -- Monooxygenases in Methanotrophs 955 -- J. Colin Murrell, Graham Stafford, and lan R. McDonald -- 53 Regulatory Responses to Copper Ions in Fungi 175 -- Simon Labbe, Jude Beaudoin, Daniel R. Bellemare, -- and Benoit Pelletier