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• W2079414750 abstract "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." @default.
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• W2079414750 date "1997-11-11" @default.
• W2079414750 modified "2023-10-11" @default.
• W2079414750 title "Degeneration of neurons, synapses, and neuropil and glial activation in a murine <i>Atm</i> knockout model of ataxia–telangiectasia" @default.
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• W2079414750 doi "https://doi.org/10.1073/pnas.94.23.12688" @default.
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