Ataxia-Telangiectasia [Paperback]

Ataxia-telangiectasia or A-T is a fatal progressiveneurological disease of children. The symptoms indicatedisruptions in the development of such diverse body parts ascerebellum, thymus and chromosomes. The patients are undulysensitive to ionizing radiation, immunodeficient, and athird of them develops cancer. All of this stems fromdefects of a single gene.Provided here is an up-to-date review of all important workin thefield. A wide spectrum of topics is covered, namelygenetics, chromosome 11 mapping, radiobiology,complementation, heterozygote identification, clinicalvariants, biochemistry, and treatment of A-T.Proceedings of the NATO Advanced Research Workshop on Ataxia-Telangiectasia, held at Newport Beach, CA, USA, May 17-20, 1992Ataxia-telangiectasia or A-T is a fatal progressiveneurological disease of children. The symptoms indicatedisruptions in the development of such diverse body parts ascerebellum, thymus and chromosomes. The patients are undulysensitive to ionizing radiation, immunodeficient, and athird of them develops cancer. All of this stems fromdefects of a single gene.Provided here is an up-to-date review of all important workin thefield. A wide spectrum of topics is covered, namelygenetics, chromosome 11 mapping, radiobiology,complementation, heterozygote identification, clinicalvariants, biochemistry, and treatment of A-T.I. Introduction.- Brief Historical Overview.- II. Isolation of A-T Gene(S).- Cloning and characterization of a candidate gene for A-T complementation Group D.- Precise localization of a gene responsible for ataxiatelangiectasia on chromosome 11q.- How many A-T genes?.- Isolation of human cDNAs that complement the ataxiatelangiectasia phenotype in cultured fibroblasts.- Complementation of the cellular A-T phenotype by gene transfer.- Use of microcell hybrids for analysis of the 11q23 region and improved localization of the A-T Group A/C genes.- AT-like radiosensitive rodent cell mutanl£*