Copper Transport and Its Disorders

1. Front Matter

   Pages i-xiii

   PDF

2. Copper Transport in Mammals

   • Maria C. Linder, Norma A. Lomeli, Stephanie Donley, Farrokh Mehrbod, Philip Cerveza, Stephen Cotton et al.

   Pages 1-16

3. Models to Evaluate Health Risks Derived from Copper Exposure/Intake in Humans

   • Manuel Olivares, Ricardo Uauy, Gloria Icaza, Mauricio González

   Pages 17-28

4. Cu Metabolism in the Liver

   • Harry J. McArdle, Michelle J. Bingham, Karl Summer, T. J. Ong

   Pages 29-37

5. Multiple Forms of the Menkes Cu-ATPase

   • Edward D. Harris, Manchi C. M. Reddy, Yongchang Qian, Evelyn Tiffany-Castiglioni, Sudeep Majumdar, John Nelson

   Pages 39-51

6. The Cell Biology of the Menkes Disease Protein

   • Michael J. Petris, Julian F. B. Mercer, James Camakaris

   Pages 53-66

7. Functional Analysis of the Menkes Protein (MNK) Expressed from a cDNA Construct

   • Sharon La Fontaine, Stephen D. Firth, Paul J. Lockhart, Hilary Brooks, James Camakaris, Julian F. B. Mercer

   Pages 67-82

8. Mutation Spectrum of ATP7A, the Gene Defective in Menkes Disease

   • Zeynep Tümer, Lisbeth Birk Møller, Nina Horn

   Pages 83-95

9. Animal Models of Menkes Disease

   • Julian F. B. Mercer, Loreta Ambrosini, Sharon Horton, Sophie Gazeas, Andrew Grimes

   Pages 97-108

10. Developmental Expression of the Mouse Mottled and Toxic Milk Genes

    • Yien-Ming Kuo, Jane Gitschier, Seymour Packman

    Pages 109-114

11. The Treatment of Wilson&’s Disease

    • George J. Brewer

    Pages 115-126

12. Indian Childhood Cirrhosis and Tyrolean Childhood Cirrhosis

    • M. S. Tanner

    Pages 127-137

13. Animal Models of Wilson’s Disease

    • J. McC. Howell

    Pages 139-152

14. Copper-Binding Properties of the N-Terminus of the Menkes Protein

    • Paul Cobine, Mark D. Harrison, Charles T. Dameron

    Pages 153-164

15. Expression, Purification, and Metal Binding Characteristics of the Putative Copper Binding Domain from the Wilson Disease Copper Transporting ATPase (ATP7B)

    • Michael DiDonato, Suree Narindrasorasak, Bibudhendra Sarkar

    Pages 165-173

16. Structure/Function Relationships in Ceruloplasmin

    • Giovanni Musci, Fabio Polticelli, Lilia Calabrese

    Pages 175-182

17. Autoxidation of Amyloid Precursor Protein and Formation of Reactive Oxygen Species

    • G. Multhaup, L. Hesse, T. Borchardt, Thomas Ruppert, R. Cappai, C. L. Masters et al.

    Pages 183-192

18. Copper-Zinc Superoxide Dismutase and ALS

    • Joan Selverstone Valentine, P. John Hart, Edith Butler Gralla

    Pages 193-203

19. A Study of the Dual Role of Copper in Superoxide Dismutase as Antioxidant and Pro-Oxidant in Cellular Models of Amyotrophic Lateral Sclerosis

    • M. T. Carrì, A. Battistoni, A. Ferri, R. Gabbianelli, G. Rotilio

    Pages 205-213

20. The Effect of Copper on Tight Junctional Permeability in a Human Intestinal Cell Line (Caco-2)

    • Simonetta Ferruzza, Yula Sambuy, Giuseppe Rotilio, Maria Rosa Ciriolo, Maria Laura Scarino

    Pages 215-222

This book is a compilation of presentations at the first meeting devoted to the mo­ lecular and cellular biology of copper transport. When we first considered the possible program for the meeting, we felt that a forum to integrate the recent advances in molecular understanding of copper transport with the older knowledge of copper metabolism was needed. In addition we wished to have a strong emphasis on the diseases of copper includ­ ing the genetic diseases, Menkes and Wilson, and other possible health aspects of this met­ al seen from a molecular perspective. Overall we were very happy with the success of the meeting, and most participants were very enthusiastic. Unfortunately we were not able to obtain manuscripts from every contributor, but the selection in this book covers most of the topics discussed. The history of biological research into copper dates from the latter half of the last century when the presence of copper as a component of living systems was first noted, but it was not until the 1920s that the essential role of copper was first recognized. l. S. McHargue found that plants and animals needed copper for optimal growth and health and proposed that copper was needed for life (McHargue, 1925). Other groups soon confirmed these observations in plants. In animals the requirement of copper for hematopoiesis was discovered in 1928 (Hart et aI.

• ATP
• biology
• cell
• cell biology
• gene
• metabolism
• mutation
• protein
• research

• University of Salerno, Salerno, Italy

  Arturo Leone

• The Murdoch Institute for Research into Birth Defects, Royal Children’s Hospital, Parkville, Australia

  Julian F. B. Mercer

Policies and ethics