Long-term depression is reduced in cerebellar Purkinje cells of dystrophin-deficient mdx mice.
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.
Anderson
Jennifer L
JL
School of Medical Sciences, University of New South Wales, Randwick 2052, Sydney, NSW, Australia.
Head
Stewart I
SI
Morley
John W
JW
D000818:Animals; D002531:Cerebellum / Q000166:cytology / Q000502:physiology; D016189:Dystrophin / Q000172:deficiency* / Q000235:genetics* / Q000502:physiology; D066298:In Vitro Techniques; D036881:Long-Term Synaptic Depression / Q000235:genetics*; D051379:Mice; D008810:Mice, Inbred C57BL; D018101:Mice, Inbred mdx; D010477:Perfusion; D011689:Purkinje Cells / Q000378:metabolism / Q000502:physiology*; D009435:Synaptic Transmission / Q000235:genetics
D016189:Dystrophin