Molecular Mechanisms of Xeroderma Pigmentosum (eBook)

Sham im I. Ahma d is a Senior Lecturer at Nottingham Trent University, Nottingham, England. After obtaining his MSc from Patna University, India, and his PhD from Leicester University, England, he joined Nottingham Polytechnic which subsequently became Nottingham Trent University. For about three decades he has been working in the field of DNA damage and repair particularly on Near UV photolysis of biological compounds, production of free radicals and their implications on human health including skin cancer and xeroderma pigmentosum. Also compounds inducing double strand DNA damage, 8-methoxypsoralen +UVA, mitomycin C, and nitrogen mustard have been under investigation including their importance in psoriasis treatment and Fanconi anemia. Additional research included: thymineless death in bacteria, genetic control of nucleotides catabolism, development of anti-AIDS drug, control of microbial infections of burns, phages of thermophiles and microbial flora of Chernobyl after the accident. In 2003 he received a prestigious “Asian Jewel Award” in Britain for “Excellence in Education”. He is also the Editor of the book, Molecular Mechanisms of Fanconi Anemia, published by Landes Bioscience. Fumio Hanaoka is a Professor at the Graduate School of Frontier Biosciences, Osaka University and the Program Leader of the Solution Oriented Research for Science and Technology of the Japan Science and Technology Agency, Japan. He received his undergraduate and PhD degrees from the University of Tokyo and did his Postdoctoral at McArdle Laboratory for Cancer Research, University of Wisconsin, Madison, USA. He joined the University of Tokyo in 1980 and in 1989 moved to RIKEN Institute as the Head of the Radiation Research Laboratory. In 1995, he joined the Institute for Molecular and Cellular Biology (now known as Graduate School of Frontier Biosciences), Osaka University. His main research interests include the molecular mechanisms of DNA replication and repair in eukaryotes. He served as the President of Molecular Biology Society of Japan (2005-2007) and has been serving on several editorial boards, including Journal of Biological Chemistry and Genes to Cells.

DEDICATION 6
PREFACE 7
ABOUT THE EDITORS... 15
PARTICIPANTS 16
CONTENTS 20
ACKNOWLEDGEMENTS 25
Historical Aspects of Xeroderma Pigmentosum and Nucleotide Excision Repair 26
Clinical Features of Xeroderma Pigmentosum 35
Xeroderma Pigmentosum and Skin Cancer 44
XPA Gene, Its Product and Biological Roles 53
XPB and XPD between Transcription and DNA Repair 64
The XPE Gene of Xeroderma Pigmentosum, Its Product and Biological Roles 82
Xeroderma Pigmentosum Variant, XP- V: Its Product and Biological Roles 118
Other Proteins Interacting with XP Proteins 128
The Nucleotide Excision Repair of DNA in Human Cells and Its Association with Xeroderma Pigmentosum 138
Roles of Oxidative Stress in Xeroderma Pigmentosum 145
Xeroderma Pigmentosum: Its Overlap with Trichothiodystrophy, Cockayne Syndrome and Other Progeroid Syndromes 153
Population Distribution of Xeroderma Pigmentosum 163
Progress and Prospects of Xeroderma Pigmentosum Therapy 169
Animal Models of Xeroderma Pigmentosum 177
INDEX 186