A subgroup of spinocerebellar ataxias defective in DNA damage responses (2007)
Gueven, N., Chen, P., Nakamura, J., Becherel, O.J., Kijas, A.W., Grattan-Smith, P., ...
A subgroup of human autosomal recessive ataxias is also characterized by disturbances of eye movement or oculomotor apraxia. These include ataxia telangiectasia (AT); ataxia telangiectasia like...
Current and potential therapeutic strategies for the treatment of ataxia-telangiectasia (2007)
Lavin, MF, Gueven, N, Bottle, S, Gatti, RA
Ataxia-telangiectasia (A-T) is a rare autosomal recessive genetic disorder characterized by progressive neurodegeneration, a high risk of cancer and immunodeficiency. These patients are also...
Gueven, N., Becherel, O.J., Howe, O., Chen, P., Haince, J.F., Ouellet, M.E., ...
Several different autosomal recessive genetic disorders characterized by ataxia with oculomotor apraxia (AOA) have been identified with the unifying feature of defective DNA damage recognition and/or...
Suraweera, A., Becherel, O. J., Chen, P., Rundle, N., Woods, R., Nakamura, J., ...
A defective response to DNA damage is observed in several human autosomal recessive ataxias with oculomotor apraxia, including ataxia-telangiectasia. We report that senataxin, defective in ataxia...
Gueven, N., Luff, J., Chen, P., Hosokawa, K., Bottle, S. E., Lavin, M. F.
Mutations in the ATM gene (mutated in ataxia telangiectasia) in both humans and mice predispose to lymphoid tumors. A defect in this gene also causes neurodegeneration in humans and a less severe...
Regulation of the Atm promoter in vivo (2006)
Gueven, N, Fukao, T, Luff, J, Paterson, C, Kay, G, Kondo, N, ...
While ATM, the protein defective in the human genetic disorder ataxia-telangiectasia (A-T), is primarily activated as a preexisting protein by radiation, there is also evidence that expression of the...
Defective p53 response and apoptosis associated with an ataxia-telangiectasia-like phenotype (2006)
Gueven, N, Becherel, OJ, Birrell, G, Chen, P, DelSal, G, Carney, JP, ...
Ataxia-telangiectasia mutated (ATM), the protein defective in ataxia-telangiectasia, plays a central role in DNA damage response and signaling to cell cycle checkpoints. We describe here a cell line...
The complexity of p53 stabilization and activation (2006)
A number of proteins are activated by stress stimuli but none so spectacularly or with the degree of complexity as the tumour suppressor p53 (human p53 gene or protein). Once stabilized, p53 is...
Becherel, OJ, Gueven, N, Birrell, GW, Schreiber, V, Suraweera, A, Jakob, B, ...
The APTX gene, mutated in patients with the neurological disorder ataxia with oculomotor apraxia type 1 (AOA1), encodes a novel protein aprataxin. We describe here, the interaction and...
Live Cell Imaging of Heavy-Ion-Induced Radiation Responses by Beamline Microscopy (2005)
B. Jakob, J. H. Rudolph, N. Gueven, M. F. Lavin, G. Taucher-Scholz
Jakob, B., Rudolph, J. H., Gueven, N., Lavin, M. F. and Taucher-Scholz, G. Live Cell Imaging of Heavy-Ion-Induced Radiation Responses by Beamline Microscopy. Radiat. Res. 163, 681–690 (2005). To...
ATM signaling and genornic stability in response to DNA damage (2005)
Lavin, MF, Birrell, G, Chen, P, Kozlov, S, Scott, S, Gueven, N
DNA double strand breaks represent the most threatening lesion to the integrity of the genome in cells exposed to ionizing radiation and radiomimetic chemicals. Those breaks are recognized, signaled...
Live cell imaging of heavy-ion-induced radiation responses by beamline microscopy (2005)
Jakob, B, Rudolph, JH, Gueven, N, Lavin, ME, Taucher-Scholz, G
To study the dynamics of protein recruitment to DNA lesions, ion beams can be used to generate extremely localized DNA damage within restricted regions of the nuclei. This inhomogeneous spatial...
Truman, JP, Gueven, N, Lavin, M, Leibel, S, Kolesnick, R, Fuks, Z, ...
Treatment with the protein kinase C activator 12-O-tetradecanoylphorbol 12-acetate (TPA) enables radiation-resistant LNCaP human prostate cancer cells to undergo radiation-induced apoptosis, mediated...
ATM and cellular response to DNA damage (2005)
Lavin, MF, Kozlov, S, Gueven, N, Peng, C, Birrell, G, Chen, P, ...
Aprataxin, a novel protein that protects against genotoxic stress (2004)
Gueven, N., Becherel, O. J., Kijas, A. W., Chen, P., Howe, O., Rudolph, J. H., ...
Ataxia-oculomotor apraxia (AOA1) is a neurological disorder with symptoms that overlap those of ataxia-telangiectasia, a syndrome characterized by abnormal responses to double-strand DNA breaks and...
Functional consequences of sequence alterations in the ATM gene (2004)
Lavin, MF, Scott, S, Gueven, N, Kozlov, S, Cheng, PA, Chen, P
The product of the gene (ATM) mutated in the human genetic disorder ataxia-telangiectasia (A-T) is a high molecular weight, protein (similar to350 kDa) containing a C-terminal protein kinase domain...
Role of ATM in radiation signal transduction (2003)
Lavin, M. F., Scott, S., Chen, P., Kozlov, S., Gueven, N., Birrell, G.
Low dose ionizing radiation-induced activation of connexin 43 expression (2003)
Glover, D, Little, JB, Lavin, MF, Gueven, N
Purpose: Connexin 43 has been implicated in the cellular response to ionizing radiation by enabling cell-to-cell communication. It is established here that the expression of connexin 43 is affected...
Gueven, N, Keating, K, Fukao, T, Loeffler, H, Kondo, N, Rodemann, HP, ...
Although ATM, the protein defective in ataxia-telangiectasia (A-T), is activated primarily by radiation, there is also evidence that expression of the protein can be regulated by both radiation and...
Kozlov, S, Gueven, N, Keating, K, Ramsay, J, Lavin, MF
Ataxia-telangiectasia Mutated (ATM), mutated in the human disorder ataxia-telangiectasia, is rapidly activated by DNA double strand breaks. The mechanism of activation remains unresolved, and it is...
Beamish, H, Kedar, P, Kaneko, H, Chen, P, Fukao, T, Peng, C, ...
Chromosome aberrations, genomic instability, and cancer predisposition are hallmarks of a number of syndromes in which the defective genes recognize and/or repair DNA damage or are involved in some...
Gueven, N, Keating, KE, Chen, P, Fukao, T, Khanna, KK, Watters, D, ...
Epidermal growth factor (EGF) has been reported to either sensitize or protect cells against ionizing radiation. We report here that EGF increases radiosensitivity in both human fibroblasts and...
Keating, KE, Gueven, N, Watters, D, Rodemann, HP, Lavin, MF
There is evidence that ATM plays a wider role in intracellular signalling in addition to DNA damage recognition and cell cycle control, In this report we show that activation of the EGF receptor is...