Main description:

Edward J. Massaro and a panel of leading biomedical researchers and clinical practitioners review, in-depth, the status of our current knowledge concerning the biochemistry of copper in general and its role in health and disease in particular. Drawing on the wealth of new information emerging from the molecular biology revolution, these experts survey the most important research areas of copper pharmacology and toxicology, including copper proteins and transport, copper toxicity and therapeutics, and copper metabolism and homeostasis. They also discuss the molecular pathogenesis of copper in a variety of metabolic diseases, Menkes and Wilson's diseases and occipital horn syndrome, as well as the role of copper in Parkinson's disease, prion disease, familial amytrophic lateral sclerosis (ALS), and Alzheimer's disease.

Contents:

Part I. Copper in Mammals

Biochemistry and Molecular Biology of Copper in Mammals
Maria C. Linder

Part II. Copper Proteins

Biochemistry of the Wilson's Disease Protein
Svetlana Lutsenko, Ruslan Tsivkovskii, Matthew J. Cooper, Brian C. MacArthur, and Hans-Peter Bachinger

Biologically Relevant Properties of Copper-Containing Proteins Analyzed by Means of Semiquantitative and Quantitative Theoretical Descriptors, Francesca De Rienzo, R. Razif Gabdoulline, Rebecca C. Wade, and M. Cristina Menziani

Molecular Modeling and Dynamics of Copper Proteins
Mattia Falconi and Alessandro Desideri

The Prion Protein and Copper: What Is the Connection?
Lesley R. Brown and David A. Harris

Prion Protein: A Synaptic Cuproprotein
David R. Brown

Cytochrome-c Oxidase
Shinya Yoshikawa

The Fet3 Protein: A Multicopper Ferroxidase Essential to Iron Metabolism in Yeast
Daniel J. Kosman

Interaction of Copper-Binding Proteins from Enteroccus hirae
Paul A. Cobine, Christopher E. Jones, Wasantha A. Wickramasinghe, Marc Solioz, and Charles T. Dameron

Part III. Copper Transport

Copper Uptake in Eukaryotic Cells
Lisbeth Birk Moller and Nina Horn

The Role of PINA in Copper Transport, Circadian Rhythms, and Wilson's Disease
Jimo Borjigin, Xing Sun, and Michael M. Wang

The Identification of Motifs Involved in the Intracellular Trafficking of the Menkes Disease Protein
Michael J. Francis and Anthony P. Monaco

Intracellular Copper Transport and ATP7B, the Wilson's Disease Protein
Kunihiko Terada and Toshihiro Sugiyama

A Multicopper Oxidase-Based Iron-Transport System in Yeast
Jerry Kaplan

Part IV. Molecular Pathogenesis of Diseases of Copper Metabolism

Molecular Basis of Diseases of Copper Homeostasis
Julian F. B. Mercer, David Kramer, andJames Camakaris

Disturbances of Copper Homeostasis and Brain Function
Giuseppe Rotilio, Maria Rosa Ciriolo, Maria Teresa Carri, and Luisa Rossi

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
Gerd Multhaup, Hermann H. Dieter, Konrad Beyreuther, and Thomas A. Bayer

Molecular Genetics and Clinical Aspects of Menkes Disease and Occipital Horn Syndrome
Hiroko Kodama

The Meaning of Alternative Transcripts of the Menkes Disease Gene
Edward D. Harris, Manchi C. M. Reddy, and Sudeep Majumdar

Expression Profiling in Menkes Disease
Zeynep Tumer, Nina Horn, and Lars Riff Jensen

Regulation by Copper of the Expression of Human Bis, a New Gene Involved in the Cellular Stress Response to Metals
Maria Gabriella Pagliuca, Rosa Lerose, and Arturo Leone

Non-Indian Childhood Cirrhosis: Using a Founder Population to Identify the Underlying Genetic Defect
Cisca Wijmenga

Part V. Copper Toxicity and Therapeutics

Biological Effects of Chronic Copper Exposure
Magdalena Araya, Mauricio Gonzalez, Manuel Olivares, and Ricardo Uauy

Copper Toxicity to Tight Junctions in the Human Intestinal Caco-2 Cell Line
Simonetta Ferruzza, Yula Sambuy, Andrea Onetti-Muda, Fabio Nobili, and Maria Laura Scarino

Cellular Responses to Copper in Aquatic Organisms: Importance of Oxidative Stress and Alteration of Signal Transduction
Aldo Viarengo, Bruno Burlando, and Claudia Bolognesi

Selective Removal of Copper Accumulating in the Form Bound to Metallothionein by Tetrathiomolybdate
Kazuo T. Suzuki and Yasumitsu Ogra

Control of Copper in Wilson's Disease and Diseases of Neovascularization, such as Cancer
George J. Brewer

Copper-Rich Metallothionein Polymers During the Development of