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. Crown Copyright (C) 2004 Published by Elsevier B.V. All rights reserved.