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Degeneration of neurons, synapses, and neuropil and glial activation in a murine Atm knockout model of ataxia-telangiectasia

Kuljis, Rodrigo O. and Xu, Yang and Aguila, M. Cecilia and Baltimore, David (1997) Degeneration of neurons, synapses, and neuropil and glial activation in a murine Atm knockout model of ataxia-telangiectasia. Proceedings of the National Academy of Sciences of the United States of America, 94 (23). pp. 12688-12693. ISSN 0027-8424. PMCID PMC25086. doi:10.1073/pnas.94.23.12688.

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Neural degeneration is one of the clinical manifestations of ataxia-telangiectasia, a disorder caused by mutations in the Atm protein kinase gene, However, neural degeneration was not detected with general purpose light microscopic methods in previous studies using several different lines of mice with disrupted Atm genes. Here, we show electron microscopic evidence of degeneration of several different types of neurons in the cerebellar cortex of 2-month-old Atm knockout mice, which is accompanied by glial activation, deterioration of neuropil structure, and both pre-and postsynaptic degeneration, These findings are similar to those in patients with ataxia-telangiectasia, indicating that Atm knockout mice are a useful model to elucidate the mechanisms underlying neurodegeneration in this condition and to develop and test strategies to palliate and prevent the disease.

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Baltimore, David0000-0001-8723-8190
Additional Information:© 1997 National Academy of Sciences. Contributed by David Baltimore, September 12, 1997. We thank Dr. Galina Olennikova and Mrs. Juanita Johnson for contributing their skill in tissue processing. We also are grateful to Drs. Nancy S. Peress (S.U.N.Y. at Stony Brook), Carol K. Petito (University of Miami), Michael Norenberg (University of Miami), and Pasko Rakic (Yale University) for critical comments on drafts of this report. This work was supported by the A-T Children’s Project, the United States Department of Veterans Affairs Merit Review Award 5065.01, the South Florida Veterans Affairs Foundation for Research and Education, National Institutes of Health and Public Health Service Grant NS 29856, and educational grants from the Eisai Corporation and Pfizer, Inc. The publication costs of this article were defrayed in part by page charge payment. This article must therefore be hereby marked “advertisement” in accordance with 18 U.S.C. §1734 solely to indicate this fact.
Funding AgencyGrant Number
Ataxia TelangiectasiaUNSPECIFIED
Department of Veterans Affairs5065.01
South Florida Veterans Affairs Foundation for Research and EducationUNSPECIFIED
NIHNS 29856
Eisai CorporationUNSPECIFIED
Subject Keywords:deficient mice, lymphoma, defects
Issue or Number:23
PubMed Central ID:PMC25086
Record Number:CaltechAUTHORS:KULpnas97
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Usage Policy:No commercial reproduction, distribution, display or performance rights in this work are provided.
ID Code:876
Deposited By: Tony Diaz
Deposited On:03 Nov 2005
Last Modified:08 Nov 2021 19:05

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