|Pleiotropic defects in ataxia-telangiectasia protein-deficient mice.|
|PMID: 8917548 Owner: NLM Status: MEDLINE|
|We have generated a mouse model for ataxia-telangiectasia by using gene targeting to generate mice that do not express the Atm protein. Atm-deficient mice are retarded in growth, do not produce mature sperm, and exhibit severe defects in T cell maturation while going on to develop thymomas. Atm-deficient fibroblasts grow poorly in culture and display a high level of double-stranded chromosome breaks. Atm-deficient thymocytes undergo spontaneous apoptosis in vitro significantly more than controls. Atm-deficient mice then exhibit many of the same symptoms found in ataxia-telangiectasia patients and in cells derived from them. Furthermore, we demonstrate that the Atm protein exists as two discrete molecular species, and that loss of one or of both of these can lead to the development of the disease.|
|A Elson; Y Wang; C J Daugherty; C C Morton; F Zhou; J Campos-Torres; P Leder|
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|Type: Journal Article|
|Title: Proceedings of the National Academy of Sciences of the United States of America Volume: 93 ISSN: 0027-8424 ISO Abbreviation: Proc. Natl. Acad. Sci. U.S.A. Publication Date: 1996 Nov|
|Created Date: 1996-12-30 Completed Date: 1996-12-30 Revised Date: 2013-04-17|
Medline Journal Info:
|Nlm Unique ID: 7505876 Medline TA: Proc Natl Acad Sci U S A Country: UNITED STATES|
|Languages: eng Pagination: 13084-9 Citation Subset: IM|
|Department of Genetics, Howard Hughes Medical Institute, Harvard Medical School, Boston, MA 02115, USA.|
|APA/MLA Format Download EndNote Download BibTex|
Ataxia Telangiectasia / genetics*
Cell Cycle Proteins
Mice, Inbred C57BL
Proteins / genetics*
Seminiferous Tubules / pathology
Spleen / immunology
T-Lymphocytes / immunology
Thymus Gland / immunology
Tumor Suppressor Proteins
|0/Cell Cycle Proteins; 0/DNA-Binding Proteins; 0/Proteins; 0/Tumor Suppressor Proteins; EC 126.96.36.199/Protein-Serine-Threonine Kinases; EC 188.8.131.52/ataxia telangiectasia mutated protein|
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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