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Copper Transport and Its Disorders : Molecular and Cellular Aspects - Arturo Leone

Copper Transport and Its Disorders

Molecular and Cellular Aspects

By: Arturo Leone (Editor), Julian F.B. Mercer (Editor)

Hardcover Published: 31st January 1999
ISBN: 9780306460456
Number Of Pages: 269

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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.

Copper Transport in Mammalsp. 1
Models to Evaluate Health Risks Derived from Copper Exposure/Intake in Humansp. 17
Cu Metabolism in the Liverp. 29
Multiple Forms of the Menkes Cu-ATPasep. 39
The Cell Biology of the Menkes Disease Proteinp. 53
Functional Analysis of the Menkes Protein (MNK) Expressed from a cDNA Constructp. 67
Mutation Spectrum of ATP7A, the Gene Defective in Menkes Diseasep. 83
Animal Models of Menkes Diseasep. 97
Developmental Expression of the Mouse Mottled and Toxic Milk Genesp. 109
The Treatment of Wilson's Diseasep. 115
Indian Childhood Cirrhosis and Tyrolean Childhood Cirrhosis: Disorders of a Copper Transport Gene?p. 127
Animal Models of Wilson's Diseasep. 139
Copper-Binding Properties of the N-Terminus of the Menkes Proteinp. 153
Expression, Purification, and Metal Binding Characteristics of the Putative Copper Binding Domain from the Wilson Disease Copper Transporting ATPase (ATP7B)p. 165
Structure/Function Relationships in Ceruloplasminp. 175
Autoxidation of Amyloid Precursor Protein and Formation of Reactive Oxygen Speciesp. 183
Copper-Zinc Superoxide Dismutase and ALSp. 193
A Study of the Dual Role of Copper in Superoxide Dismutase as Antioxidant and Pro-Oxidant in Cellular Models of Amyotrophic Lateral Sclerosisp. 205
The Effect of Copper on Tight Junctional Permeability in a Human Intestinal Cell Line (Caco-2)p. 215
Metal Regulation of Metallothionein Gene Transcription in Mammalsp. 223
Copper-Regulatory Domain Involved in Gene Expressionp. 237
Intracellular Pathways of Copper Trafficking in Yeast and Humansp. 247
Copper Homeostasis in Enterococcus hiraep. 255
Indexp. 265
Table of Contents provided by Syndetics. All Rights Reserved.

ISBN: 9780306460456
ISBN-10: 0306460459
Series: Advances in Experimental Medicine and Biology
Audience: Professional
Format: Hardcover
Language: English
Number Of Pages: 269
Published: 31st January 1999
Publisher: Springer Science+Business Media
Country of Publication: US
Dimensions (cm): 23.5 x 15.5  x 1.91
Weight (kg): 1.27