Mouse models as a tool for understanding neurodegenerative diseases

被引:12
作者
Ahmad-Annuar, A [1 ]
Tabrizi, SJ [1 ]
Fisher, EMC [1 ]
机构
[1] UCL Natl Hosp Neurol & Neurosurg, Inst Neurol, Dept Neurodegenerat Dis, London WC1N 3BG, England
关键词
mouse models; transgenics and neurodegenerative disease; knockouts/gene targeting and neurodegenerative disease;
D O I
10.1097/01.wco.0000084221.82329.29
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Purpose of review The purpose of this review is to present recent advances in the both the creation and the use of mouse models of human neurodegenerative disease. We briefly touch on the technologies used to make these models, and then focus on recent results from new models. We discuss why such models are useful when they do - and do not - mimic the human disorder. Recent findings The numbers of mouse models are increasing dramatically and are starting to yield important results for human disease. We present a selection of new and important models and the results of recent investigations of these animals. Summary An accepted protocol when studying any form of human neurodegenerative disease is to investigate the genetics, pathology, neurophysiology, response to therapeutics, etc., of the disorder in the mouse. This approach is clearly bearing fruit for our understanding and treatment of human neurodegeneration.
引用
收藏
页码:451 / 458
页数:8
相关论文
共 70 条
[1]  
BATES GP, 2002, MOUSE MODELS HUNTING, P387
[2]   Missense mutation in the tubulin-specific chaperone E (Tbce) gene in the mouse mutant progressive motor neuronopathy, a model of human motoneuron disease [J].
Bömmel, H ;
Xie, G ;
Rossoll, W ;
Wiese, S ;
Jablonka, S ;
Boehm, T ;
Sendtner, M .
JOURNAL OF CELL BIOLOGY, 2002, 159 (04) :563-569
[3]   Mutations in the DJ-1 gene associated with autosomal recessive early-onset parkinsonism [J].
Bonifati, V ;
Rizzu, P ;
van Baren, MJ ;
Schaap, O ;
Breedveld, GJ ;
Krieger, E ;
Dekker, MCJ ;
Squitieri, F ;
Ibanez, P ;
Joosse, M ;
van Dongen, JW ;
Vanacore, N ;
van Swieten, JC ;
Brice, A ;
Meco, G ;
van Duijn, CM ;
Oostra, BA ;
Heutink, P .
SCIENCE, 2003, 299 (5604) :256-259
[4]   Accelerated amyloid deposition in the brains of transgenic mice coexpressing mutant presenilin 1 and amyloid precursor proteins [J].
Borchelt, DR ;
Ratovitski, T ;
vanLare, J ;
Lee, MK ;
Gonzales, V ;
Jenkins, NA ;
Copeland, NG ;
Price, DL ;
Sisodia, SS .
NEURON, 1997, 19 (04) :939-945
[5]   Mice with mutations in the mahogany gene Atrn have cerebral spongiform changes [J].
Bronson, RT ;
Donahue, LR ;
Samples, R ;
Naggert, JK .
JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY, 2001, 60 (07) :724-730
[6]   Amyotrophic lateral sclerosis - Insights from genetics [J].
Brown, RH .
ARCHIVES OF NEUROLOGY, 1997, 54 (10) :1246-1250
[7]   Systematic approaches to mouse mutagenesis [J].
Brown, SDM ;
Balling, R .
CURRENT OPINION IN GENETICS & DEVELOPMENT, 2001, 11 (03) :268-273
[8]   Null mutation of the murine ATP7B (Wilson disease) gene results in intracellular copper accumulation and late-onset hepatic nodular transformation [J].
Buiakova, OI ;
Xu, J ;
Lutsenko, S ;
Zeitlin, S ;
Das, K ;
Das, S ;
Ross, BM ;
Mekios, C ;
Scheinberg, IH ;
Gilliam, TC .
HUMAN MOLECULAR GENETICS, 1999, 8 (09) :1665-1671
[9]   SCA1 TRANSGENIC MICE - A MODEL FOR NEURODEGENERATION CAUSED BY AN EXPANDED CAG TRINUCLEOTIDE REPEAT [J].
BURRIGHT, EN ;
CLARK, HB ;
SERVADIO, A ;
MATILLA, T ;
FEDDERSEN, RM ;
YUNIS, WS ;
DUVICK, LA ;
ZOGHBI, HY ;
ORR, HT .
CELL, 1995, 82 (06) :937-948
[10]   YAC transgenic mice carrying pathological alleles of the MJD1 locus exhibit a mild and slowly progressive cerebellar deficit [J].
Cemal, CK ;
Carroll, CJ ;
Lawrence, L ;
Lowrie, MB ;
Ruddle, P ;
Al-Mahdawi, S ;
King, RHM ;
Pook, MA ;
Huxley, C ;
Chamberlain, S .
HUMAN MOLECULAR GENETICS, 2002, 11 (09) :1075-1094