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Atm-deficient mice: a paradigm of ataxia telangiectasia.

A murine model of ataxia telangiectasia was created by disrupting the Atm locus via gene targeting. Mice homozygous for the disrupted Atm allele displayed growth retardation, neurologic dysfunction, male and female infertility secondary to the absence of mature gametes, defects in T lymphocyte maturation, and extreme sensitivity to gamma-irradiation. The majority of animals developed malignant thymic lymphomas between 2 and 4 months of age. Several chromosomal anomalies were detected in one of these tumors. Fibroblasts from these mice grew slowly and exhibited abnormal radiation-induced G1 checkpoint function. Atm-disrupted mice recapitulate the ataxia telangiectasia phenotype in humans, providing a mammalian model in which to study the pathophysiology of this pleiotropic disorder.

Pubmed ID: 8689683

Authors

  • Barlow C
  • Hirotsune S
  • Paylor R
  • Liyanage M
  • Eckhaus M
  • Collins F
  • Shiloh Y
  • Crawley JN
  • Ried T
  • Tagle D
  • Wynshaw-Boris A

Journal

Cell

Publication Data

July 12, 1996

Associated Grants

None

Mesh Terms

  • Animals
  • Ataxia Telangiectasia
  • Ataxia Telangiectasia Mutated Proteins
  • Cell Cycle
  • Cell Cycle Proteins
  • Cell Division
  • DNA-Binding Proteins
  • Disease Models, Animal
  • Dose-Response Relationship, Radiation
  • Female
  • Fibroblasts
  • Germ Cells
  • Leucine Zippers
  • Lymphoma
  • Male
  • Mice
  • Mice, Mutant Strains
  • Mutation
  • Neurologic Examination
  • Protein-Serine-Threonine Kinases
  • Proteins
  • Thymus Neoplasms
  • Tumor Suppressor Proteins