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

Jennifer L. Anderson; Stewart I. Head; John W. Morley

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

Jennifer L. Anderson, Stewart I. Head, John W. Morley

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Abstract

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.

Original language	English
Number of pages	4
Journal	Brain research
Publication status	Published - 2004

Keywords

duchenne
muscular dystrophy
synaptic plasticity

Cite this

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title = "Long-term depression is reduced in cerebellar Purkinje cells of dystrophin-deficient mdx mice",

abstract = "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.",

keywords = "duchenne, muscular dystrophy, synaptic plasticity",

author = "Anderson, {Jennifer L.} and Head, {Stewart I.} and Morley, {John W.}",

year = "2004",

language = "English",

journal = "Brain research",

issn = "0259-0819",

publisher = "Elsevier",

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T1 - Long-term depression is reduced in cerebellar Purkinje cells of dystrophin-deficient mdx mice

AU - Anderson, Jennifer L.

AU - Head, Stewart I.

AU - Morley, John W.

PY - 2004

Y1 - 2004

N2 - 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.

AB - 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.

KW - duchenne

KW - muscular dystrophy

KW - synaptic plasticity

UR - http://handle.uws.edu.au:8081/1959.7/9680

M3 - Article

SN - 0259-0819

JO - Brain research

JF - Brain research

ER -

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

Abstract

Keywords

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