The hippocampal form of an essential muscle protein called dystrophin is found in higher levels in people with temporal lobe epilepsy (TLE), according to a new study published in the scientific journal Frontiers in Cellular Neuroscience.This could be the result of a compensatory mechanism – in response to too much excitation in the brain (hyperexcitation) – that tries to restore the inhibitory balance. Exploring the relationship between the different forms of dystrophin found in the central nervous system and hyperexcitation may highlight a new treatment target for epileptic seizures.The researchers, led by Dr Johan Vles, at Maastricht University Medical Centre in the Netherlands, took biopsies from the brains of people with TLE and looked at the distribution of the dystrophin protein within them. They compared this to similar samples obtained from deceased donors who did not have epilepsy (controls).They found that the level of full-length dystrophin in the hippocampus (an important memory structure) was 60% higher in people with TLE than in controls.The team then looked at the levels of dystrophin in the hippocampus and the cerebellum (which helps regulate muscle activity) of a rat model of TLE, and compared them to control animals; however they saw no differences between the two groups in either region.Taken together, these results suggest that the increase in dystrophin in TLE is specific to the hippocampus and also unique to human epilepsy. Investigation of the mechanisms behind the increase in hippocampal dystrophin in response to hyperexcitation could lead to new therapies.Dystrophin is an essential muscle protein and mutations in the dystrophin gene that result in the absence of the protein cause a muscle-wasting condition known as Duchenne muscular dystrophy. In addition to the role it plays in muscle tissue, dystrophin also functions as an anchoring protein within the central nervous system, which explains why Duchenne muscular dystrophy may be accompanied by cognitive and behavioural difficulties and epilepsy.Author: Dr Özge ÖzkayaClick here for more articles about brain science including genetics.
August 9th, 2016|