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Produktbild: Molecular Mechanisms of Xeroderma Pigmentosum
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Molecular Mechanisms of Xeroderma Pigmentosum

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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.

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Erscheinungsdatum

30.11.2008

Herausgeber

Shamim Ahmad + weitere

Verlag

Springer Us

Seitenzahl

166 (Printausgabe)

Dateigröße

23494 KB

Sprache

Englisch

EAN

9780387095998

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Erscheinungsdatum

30.11.2008

Herausgeber

Verlag

Springer Us

Seitenzahl

166 (Printausgabe)

Dateigröße

23494 KB

Sprache

Englisch

EAN

9780387095998

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  • Produktbild: Molecular Mechanisms of Xeroderma Pigmentosum
  • 1. Historical Aspects of Xeroderma Pigmentosum and Nucleotide Excision Repair James E. Cleaver Abstract Historical Aspects 2. Clinical Features of Xeroderma Pigmentosum Ulrich R. Hengge and Steffen Emmert Background Epidemiology Dermatological Manifestations Other Cancers in Xeroderma Pigmentosum Neurological Manifestations Ophthalmological Manifestations Differential Diagnoses Diagnosis of XP Management Prognosis 3. Xeroderma Pigmentosum and Skin Cancer Leela Daya-Grosjean Abstract Introduction XP Genetics and Skin Cancer Cutaneous Malignancies in XP Patients The Etiology of XP Skin Cancers and UV Hallmark Mutations Tumor Suppressor Genes Oncogene Activation in XP Skin Tumors Modifications of the SHH Signaling Pathway Genes in XP BCC Impaired Immune Response in XP Patients and Human Papilloma Virus Conclusions 4. XPA Gene, Its Product and Biological Roles Ulrike Camenisch and Hanspeter Nägeli Abstract Introduction XPA Gene XPA Protein and Interactions with other NER Factors DNA-Binding Activity of XPA Protein Recruitment of XPA to Active NER Complexes Role of XPA-RPA Interactions How Does XPA Protein Contribute to DNA Damage Recognition? A Hypothesis for the Mechanism of Damage Verification by XPA 5. XPB and XPD between Transcription and DNA Repair Brian D. Beck, Dae-Sik Hah and Suk-Hee Lee Introduction Structure-Function Relationship Role in Transcription Role in NER Other Roles of XPD Lessons from Genetic Variations of XPB/XPD 6. XPC: Its Product and Biological Roles Kaoru Sugasawa Abstract Introduction XPC Is a Damage Recognition Factor in GG-NER Roles for Other Subunits Ubiquitination and Interaction with UV-DDB Possible Functions of XPC beyond NER 7. The XPE gene of Xeroderma Pigmentosum, its product and biological roles Drew Bennett and Toshiki Itoh Discovery and Background Expression and Regulation of DDB protein Mouse Model Protein Interactions DNA Binding of the DDB complex Current Models of DDB function Conclusion 8. XPF/ERC4 and ERC1: Their Products and Biological Roles Lisa D. McDaniel and Roger A. Schultz Introduction XPF Patients, ERC4 Mutant Cells and Gene Cloning Mouse Models with Relevance to XPF (Ercc1 and Ercc4) XPF in Nucleotide Excision Repair ERCC4 in Immunoglobin Switching ERC4 in Crosslink Repair ERC4 in Telomeres Summary 9. XPG: Its Products and Biological Roles Orlando D. Schärer Abstract Introduction Discovery and Cloning of XPG Biochemical Properties of the XPG Protein The Role of XPG in Nucleotide Excision Repair Roles of XPG Outside of Nucleotide Excision Repair XP-G Patients and Their Mutant Alleles XP Group G Patients without CS Patients with Severe XP and CS Symptoms XP-G Patients with Late-Onset CS Symptoms Mouse Models with XPG Deficiency Conclusion 10. Xeroderma pigmentosum variant, XP-V: its product and biological roles Chikahide Masutani, Fumio Hanaoka and Shamim I. Ahmad Introduction Human DNA polymerases XP-V gene and Its homologues Structure and activities of polymerase Bypassing of unusual nucleotides by pol Mutation in POLH and Its effects Mechanism of mutagenesis in pol mutant Strains-The roles of other polymerases Interaction of Pol with Other Proteins Mouse, Plant and Microbial Models for Pol Conclusion 11. Other Proteins Interacting with XP Proteins Steven M. Shell and Yue Zou Introduction Finding DNA Damage: XPA, XPC and XPE Preparing the Site: XPB and XPD Cutting It Out: XPG and XPF Getting Past It: XPV Conclusions 12. The Nucleotide Excision Repa ir of DNA in Human Cells and Its Association with Xeroderma Pigmentosum Alexei Gratchev Introduction XP Associated Genes and Their Roles in NER NER Pathway 13. Roles of Oxidative Stress in Xeroderma Pigmentosum Masaharu Hayashi Abstract Reactive Oxygen Species and Oxidative Stress Oxidative Damage at the Cellular Level in Xeroderma Pigmentosum Oxidative Stress in Neurodegenerative Disorders Neuropathological Analysis on Oxidative Stress in the Brains of Autopsy Cases of Xeroderma Pigmentosum and Cockayne Syndrome Preliminary ELISA Analysis of Oxidative Stress Markers in Urinary Samples from Patients with Xeroderma Pigmentosum 14. Xeroderma Pigmentosum, its overlap with Trichothiodystrophy, Cockayne Syndrome and Other Progeroid Syndromes W. Clark Lambert, Claude E. Gagna and Muriel W. Lambert Introduction The Ultraviolet-Sensitive Syndrome (UVSS) XP/Trichothiodystrophy (XP/TTD) Overlap Syndromes XP-D/TTD Overlap Syndromes XP/Cockayne Syndrome (XP/CS) Overlap Syndromes XP-B/CS Overlap Syndrome(s) XP-D/CS Overlap Syndrome(s) XP-G/CS Overlap Syndrome(s) XP-H/CS Overlap Syndrome(s) XP/CSB Overlap Syndrome(s) XP/Progeroid Overlap Syndromes XP-F/Progeroid Overlap syndrome 15. Population Distribution of Xeroderma Pigmentosum Abdul Manan Bhutto and Sandra H. Kirk Introduction Population Distribution Genetics and Population Population and Malignancy Type 16. Progress and Prospects of Xeroderma Pigmentosum Therapy Alain Sarasin Introduction Protection Towards UV Exposure Classical XP Therapy Conclusions 17. Animal Models of Xeroderma Pigmentosum Xue-Zhi Sun, Rui Zhang, Chun Cui, Yoshi-Nobu Harada, Setsuji Hisano, Yeunhwa Gu, Yoshihiro Fukui and Hidenori Yonehara Introduction Xpa-Deficient Mouse Models Xpc-Deficient Mouse Models Xpg-Deficient Mouse Models Ercc1/Xpf-Deficient Mouse Models Xpd Mutant Mouse Models Xpa /Csb Mutant Mouse Models Index