Publications - Ataxia Telangiectasia

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ATM, the Mre11/RAd50/nBS1 complex, and topoisomerase I are concentrated in the nucleus of purkinje neurons in the juvenile human brain. Gorodetsky, E., Calkins, S., Ahn, J and Brooks, PJ. DNA Repair 6:1698-1707, 2007. [paper] 

Astrocyte dysfunction in ataxia telangiectasia mediates neuronal survival and defects in neuronal maturation. Bushman, J., Noble, M. and Mayer-Proschel, M. SFN, 2008. [abstract] 

Ataxia-telangiectasia mutated kinase regulates ribonucleotide reductase and mitochondrial homeostasis. Eaton, J., Lin, P., Sartorelli, A, Bonawitz, N. and Shadel, G. J of Clincal Investigation 117: 2723-2734, 2007. [paper] 

Differential expression of small heat shock protein 27 (Hsp27) in ataxia telangiectasia brains. Chen, W., Kuizon, S., Chiou, B., Bolton, D., Pullarkat, R. and Junaid, M. Neurochem. Res 34: 1658-1667, 2009. [paper] 

Increased chromosome instability dramatically disrupts neural genome integrity and mediates cerebellar degeneration in the ataxia-telangiectasia brain. Iourov, I., Vorsanova, S., Liehr, T., Kolotii nd Yurov, Y.B. Human Molecular Genetics 18: 2656-2669, 2009. [paper]