Long-term depression is reduced in cerebellar Purkinje cells of dystrophin-deficient mdx mice

被引:37
作者
Anderson, JL [1 ]
Head, SI [1 ]
Morley, JW [1 ]
机构
[1] Univ New S Wales, Sch Med Sci, Sydney, NSW 2052, Australia
关键词
muscular dystrophy; Duchenne; synaptic plasticity;
D O I
10.1016/j.brainres.2004.06.011
中图分类号
Q189 [神经科学];
学科分类号
071006 ;
摘要
The mdx (muscular dystrophy X-linked) mouse is a model for human Duchenne muscular dystrophy (DMD) and is characterized by the absence of the cytoskeletal protein dystrophin. Using a cerebellar slice preparation, we show that postsynaptically mediated long-term depression (LTD) is significantly reduced in mdx Purkinje cells, while presynaptically mediated paired-pulse facilitation (PPF) is normal. This disruption of LTD could contribute to the cognitive deficit in boys with Duchenne muscular dystrophy. Crown Copyright (C) 2004 Published by Elsevier B.V. All rights reserved.
引用
收藏
页码:289 / 292
页数:4
相关论文
共 18 条
[1]  
ADNERSON JL, 2003, BRAIN RES, V982, P280
[2]   REDUCED HIPPOCAMPAL LONG-TERM POTENTIATION AND CONTEXT-SPECIFIC DEFICIT IN ASSOCIATIVE LEARNING IN MGLUR1 MUTANT MICE [J].
AIBA, A ;
CHEN, C ;
HERRUP, K ;
ROSENMUND, C ;
STEVENS, CF ;
TONEGAWA, S .
CELL, 1994, 79 (02) :365-375
[3]  
Atluri PP, 1996, J NEUROSCI, V16, P5661
[4]   The neurobiology of Duchenne muscular dystrophy:: learning lessons from muscle? [J].
Blake, DJ ;
Kröger, S .
TRENDS IN NEUROSCIENCES, 2000, 23 (03) :92-99
[5]   GABAergic terminals are required for postsynaptic clustering of dystrophin but not of GABAA receptors and gephyrin [J].
Brünig, I ;
Suter, A ;
Knuesel, I ;
Lüscher, B ;
Fritschy, JM .
JOURNAL OF NEUROSCIENCE, 2002, 22 (12) :4805-4813
[6]  
Cabeza Roberto, 2000, Current Opinion in Neurology, V13, P415, DOI 10.1097/00019052-200008000-00008
[7]   The dystrophinopathies: An alternative to the structural hypothesis [J].
Carlson, CG .
NEUROBIOLOGY OF DISEASE, 1998, 5 (01) :3-15
[8]   ACTIVATION OF PROTEIN KINASE-C INDUCES A LONG-TERM DEPRESSION OF GLUTAMATE SENSITIVITY OF CEREBELLAR PURKINJE-CELLS - AN INVITRO STUDY [J].
CREPEL, F ;
KRUPA, M .
BRAIN RESEARCH, 1988, 458 (02) :397-401
[9]  
Emery A E, 1991, Neuromuscul Disord, V1, P19, DOI 10.1016/0960-8966(91)90039-U
[10]   MEMBRANE-POTENTIAL, RESTING CALCIUM AND CALCIUM TRANSIENTS IN ISOLATED MUSCLE-FIBERS FROM NORMAL AND DYSTROPHIC MICE [J].
HEAD, SI .
JOURNAL OF PHYSIOLOGY-LONDON, 1993, 469 :11-19