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A possible role of dystrophin in neuronal excitability: A review of the current literature

Hendriksen, R. G. F., Hoogland, G., Schipper, S., Hendriksen, J. G. M., Vles, J. S. H. & Aalbers, M. W., Apr-2015, In : Neuroscience and Biobehavioral Reviews. 51, p. 255-262 8 p.

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  • A possible role of dystrophin in neuronal excitability

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DOI

  • Ruben G. F. Hendriksen
  • Govert Hoogland
  • Sandra Schipper
  • Jos G. M. Hendriksen
  • Johan S. H. Vles
  • Marlien W. Aalbers

Duchenne muscular dystrophy (DMD) is a recessive hereditary form of muscular dystrophy caused by a mutation in the dystrophin gene on the X chromosome. Clinical observations show that in addition to progressive muscular degeneration, DMD is more often accompanied by neurocognitive symptoms and learning disabilities, especially in automatisation of reading, attention processes, and expressive language skills. Additionally, three studies reported a higher prevalence of epilepsy in DMD, suggesting that the absence of dystrophin might be related to increased CNS excitability. In this article, we aim to review current clinical and experimental evidence for a potential role of brain dystrophin in seizure generation. (C) 2015 Elsevier Ltd. All rights reserved.

Original languageEnglish
Pages (from-to)255-262
Number of pages8
JournalNeuroscience and Biobehavioral Reviews
Volume51
Publication statusPublished - Apr-2015
Externally publishedYes

    Keywords

  • Duchenne muscular dystrophy, Dystrophin, Epilepsy, Seizures, DUCHENNE MUSCULAR-DYSTROPHY, TEMPORAL-LOBE EPILEPSY, BLOOD-BRAIN-BARRIER, DEFICIENT MDX MICE, CEREBELLAR PURKINJE-CELLS, GABA(A) RECEPTOR NUMBER, LONG-TERM POTENTIATION, GLYCOPROTEIN COMPLEX, SEIZURE SUSCEPTIBILITY, SYNAPTIC PLASTICITY

ID: 130079991