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.
School of Medical Sciences, University of New South Wales, Randwick 2052, Sydney, NSW, Australia.
Jennifer L
JL
Anderson
Stewart I
SI
Head
John W
JW
Morley
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