Linking DNA damage and neurodegeneration

被引:140
作者
Rolig, RL [1 ]
McKinnon, PJ [1 ]
机构
[1] St Jude Childrens Res Hosp, Dept Genet, Memphis, TN 38105 USA
关键词
D O I
10.1016/S0166-2236(00)01625-8
中图分类号
Q189 [神经科学];
学科分类号
071006 ;
摘要
Many human pathological conditions with genetic defects in DNA damage responses are also characterized by neurological deficits. These neurological deficits can manifest themselves during many stages of development, suggesting an important role for DNA repair or processing during the development and maintenance of the nervous system. Although the molecular neuropathology associated with such deficits is largely unknown, many of the responsible gene defects have been identified. The current rapid progress in elucidation of molecular details following gene identification should provide further insight into the importance of DNA processing in nervous system function.
引用
收藏
页码:417 / 424
页数:8
相关论文
共 105 条
[1]   FANCONI-ANEMIA [J].
AUERBACH, AD .
DERMATOLOGIC CLINICS, 1995, 13 (01) :41-49
[2]   The Werner syndrome protein is involved in RNA polymerase II transcription [J].
Balajee, AS ;
Machwe, A ;
May, A ;
Gray, MD ;
Oshima, J ;
Martin, GM ;
Nehlin, JO ;
Brosh, R ;
Orren, DK ;
Bohr, VA .
MOLECULAR BIOLOGY OF THE CELL, 1999, 10 (08) :2655-2668
[3]   Loss of the ataxia-telangiectasia gene product causes oxidative damage in target organs [J].
Barlow, C ;
Dennery, PA ;
Shigenaga, MK ;
Smith, MA ;
Morrow, JD ;
Roberts, LJ ;
Wynshaw-Boris, A ;
Levine, RL .
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1999, 96 (17) :9915-9919
[4]   Targeted disruption of the gene encoding DNA ligase IV leads to lethality in embryonic mice [J].
Barnes, DE ;
Stamp, G ;
Rosewell, I ;
Denzel, A ;
Lindahl, T .
CURRENT BIOLOGY, 1998, 8 (25) :1395-1398
[5]  
Bohr V A, 1998, J Investig Dermatol Symp Proc, V3, P11
[6]  
BOOTSMA D, 1991, ANN GENET-PARIS, V34, P143
[7]   Analysis of mutations in the XPD gene in Italian patients with trichothiodystrophy:: Site of mutation correlates with repair deficiency, but gene dosage appears to determine clinical severity [J].
Botta, E ;
Nardo, T ;
Broughton, BC ;
Marinoni, S ;
Lehmann, AR ;
Stefanini, M .
AMERICAN JOURNAL OF HUMAN GENETICS, 1998, 63 (04) :1036-1048
[8]  
Brooks PJ, 1996, J NEUROSCI, V16, P939
[9]   Detection of excision nuclease in cell-free extracts from the adult mammalian brain [J].
Brooks, PJ .
MUTATION RESEARCH-DNA REPAIR, 1998, 408 (01) :37-46
[10]   Functional and physical interaction between WRN helicase and human replication protein A [J].
Brosh, RM ;
Orren, DK ;
Nehlin, JO ;
Ravn, PH ;
Kenny, MK ;
Machwe, A ;
Bohr, VA .
JOURNAL OF BIOLOGICAL CHEMISTRY, 1999, 274 (26) :18341-18350