Defective DNA Repair and Neurodegenerative Disease

by: Ulrich Rass, Ivan Ahel, Stephen C West

Cell, Vol. 130, No. 6. (21 September 2007), pp. 991-1004.

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Abstract

Defects in cellular DNA repair processes have been linked to genome instability, heritable cancers, and premature aging syndromes. Yet defects in some repair processes manifest themselves primarily in neuronal tissues. This review focuses on studies defining the molecular defects associated with several human neurological disorders, particularly ataxia with oculomotor apraxia 1 (AOA1) and spinocerebellar ataxia with axonal neuropathy 1 (SCAN1). A picture is emerging to suggest that brain cells, due to their nonproliferative nature, may be particularly prone to the progressive accumulation of unrepaired DNA lesions.

@article{citeulike:3036972, abstract = {Defects in cellular DNA repair processes have been linked to genome instability, heritable cancers, and premature aging syndromes. Yet defects in some repair processes manifest themselves primarily in neuronal tissues. This review focuses on studies defining the molecular defects associated with several human neurological disorders, particularly ataxia with oculomotor apraxia 1 (AOA1) and spinocerebellar ataxia with axonal neuropathy 1 (SCAN1). A picture is emerging to suggest that brain cells, due to their nonproliferative nature, may be particularly prone to the progressive accumulation of unrepaired DNA lesions.}, author = {Rass, Ulrich and Ahel, Ivan and West, Stephen C. }, citeulike-article-id = {3036972}, doi = {http://dx.doi.org/10.1016/j.cell.2007.08.043}, journal = {Cell}, keywords = {aprataxin, neurologicdiseases, xrcc4}, month = {September}, number = {6}, pages = {991--1004}, posted-at = {2008-07-23 15:27:18}, priority = {2}, title = {Defective DNA Repair and Neurodegenerative Disease}, url = {http://dx.doi.org/10.1016/j.cell.2007.08.043}, volume = {130}, year = {2007} }

RIS record

TY - JOUR ID - citeulike:3036972 L3 - citeulike-article-id:3036972 TI - Defective DNA Repair and Neurodegenerative Disease JF - Cell VL - 130 IS - 6 SP - 991 EP - 1004 N2 - Defects in cellular DNA repair processes have been linked to genome instability, heritable cancers, and premature aging syndromes. Yet defects in some repair processes manifest themselves primarily in neuronal tissues. This review focuses on studies defining the molecular defects associated with several human neurological disorders, particularly ataxia with oculomotor apraxia 1 (AOA1) and spinocerebellar ataxia with axonal neuropathy 1 (SCAN1). A picture is emerging to suggest that brain cells, due to their nonproliferative nature, may be particularly prone to the progressive accumulation of unrepaired DNA lesions. KW - aprataxin KW - neurologicdiseases KW - xrcc4 AU - Rass, Ulrich AU - Ahel, Ivan AU - West, Stephen PY - 2007/09/21/ UR - http://dx.doi.org/10.1016/j.cell.2007.08.043 ER -

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