Ataxia-Telangiectasia

1. Front Matter

   Pages I-XXIII

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2. Introduction

   1. Front Matter

      Pages XIX-XIX

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   2. Ataxia-Telangiectasia: A Brief Historical Overview

      • Robert P. Sedgwick

      Pages 1-3

3. Isolation of A-T Gene(s)

   1. Front Matter

      Pages 5-5

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   2. Cloning and Characterization of a Candidate Gene for A-T Complementation Group D

      • Leon N. Kapp, John P. Murnane

      Pages 7-22

   3. Precise Localization of a Gene Responsible for Ataxia-Telangiectasia on Chromosome 11q

      • F. Cornélis, M. James, D. Cherif, T. Tokino, J. Davies, D. Girault et al.

      Pages 23-35

   4. How Many Ataxia-Telangiectasia Genes?

      • Ethan Lange, Richard A. Gatti, Eric Sobel, Patrick Concannon, Kenneth Lange

      Pages 37-54

   5. Isolation of Human cDNAS That Complement the Ataxia-Telangiectasia Phenotype in Cultured Fibroblasts

      • M. Stephen Meyn, Jennifer M. Lu-Kuo, Laura B. K. Herzing

      Pages 55-64

   6. Complementation of the Cellular A-T Phenotype by Gene Transfer

      • Yael Ziv, Tsafi Danieli, Galit Rotman, Adam Sartiel, Anat Bar-Shira, N. G. J. Jaspers et al.

      Pages 65-74

   7. Use of Microcell Hybrids for Analysis of the 11q23 Region and Improved Localization of the A-T Group A/C Genes

      • Y. Ejima, M. Oshimura, M. S. Sasaki

      Pages 75-85

   8. AT-like Radiosensitive Rodent Cell Mutants: An Alternative Approach to the Isolation of the A-T Gene(s)

      • M. Z. Zdzienicka, G. W. C. T. Verhaegh, W. Jongmans, N. G. J. Jaspers, M. Oshimura, M. R. James et al.

      Pages 87-97

4. A-T Heterozygotes and Complementation

   1. Front Matter

      Pages 99-99

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   2. Identification of A-T heterozygotes

      • D. Scott, L. A. Jones, S. A. G. Elyan, A. Spreadborough, R. Cowan, G. Ribiero

      Pages 101-116

   3. Correction of Post-γ Ray DNA Repair Deficiency in Ataxia-Telangiectasia Complementation Group A Fibroblasts by Cocultivation with Normal Fibroblasts

      • M. C. Paterson, R. Mirzayans

      Pages 117-126

   4. The A-T gene does not make a major contribution to familial breast cancer

      • Richard Wooster, Douglas F. Easton, Deborah Ford, Jonathan Mangion, Bruce A. J. Ponder, Julian Peto et al.

      Pages 127-135

   5. Mammography Screening for A-T Heterozygotes

      • Amos Norman, H. Rodney Withers

      Pages 137-140

5. Defining the A-T Defect

   1. Front Matter

      Pages 141-141

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   2. Lymphoid V(D)J Recombination: Accessibility and Reaction Fidelity in Normal and Ataxia-Telangiectasia Cells

      • Chih-Lin Hsieh, Michael R. Lieber

      Pages 143-154

   3. Murine scid cells and human ataxia-telangiectasia cells complement each other’s radiosensitivity

      • Kenshi Komatsu

      Pages 155-161

   4. Ataxia-Telangiectasia: Defective in a P53-Dependent Signal Transduction Pathway

      • Michael B. Kastan

      Pages 163-173

Ataxia-telangiectasia or A-T is a fatal progressive neurological disease of children. The symptoms indicate disruptions in the development of such diverse body parts as cerebellum, thymus and chromosomes. The patients are unduly sensitive to ionizing radiation, immunodeficient, and a third of them develops cancer. All of this stems from defects of a single gene. Provided here is an up-to-date review of all important work in thefield. A wide spectrum of topics is covered, namely genetics, chromosome 11 mapping, radiobiology, complementation, heterozygote identification, clinical variants, biochemistry, and treatment of A-T.

• Ataxia-Telangiectasia
• Cancer Research
• Cerebellar Ataxia
• Chromosom
• Gendefekt
• Gene Defect
• Immunodeficiency
• Immunschwäche
• Krebsforschung
• Neuropathologie
• Radiosensitivity
• Strahlenempfindlichkeit
• genetics

• Department of Pathology, University of California Los Angeles School of Medicine, Los Angeles, USA

  Richard A. Gatti

• Laboratory of Radiobiology and Environmental Health, University of California San Francisco, San Francisco, USA

  Robert B. Painter

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