A possible role of dystrophin in neuronal excitability: A review of the current literatureHendriksen, 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.
Research output: Contribution to journal › Review article › Academic › peer-review
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.
|Number of pages||8|
|Journal||Neuroscience and Biobehavioral Reviews|
|Publication status||Published - Apr-2015|
- 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