title: Mitochondrial involvement in temporal lobe epilepsy.
abstract: Mitochondrial dysfunction has been identified as a potential cause of epileptic seizures and therapy-resistant forms of severe epilepsy. Thus, a broad variety of mutation in mitochondrial DNA or nuclear genes leading to the impairment of mitochondrial respiratory chain or of mitochondrial ATP synthesis has been associated with epileptic phenotypes. Additionally, with a variety of different methods impaired mitochondrial function has been reported for the seizure focus of patients with temporal lobe epilepsy and Ammon's horn sclerosis and of animal models of temporal lobe epilepsy. Since mitochondrial oxidative phosphorylation provides the major source of ATP in neurons and mitochondria participate in cellular Ca(2+) homeostasis, their dysfunction strongly affects neuronal excitability and synaptic transmission, which is proposed to be highly relevant for seizure generation. Additionally, mitochondrial dysfunction is known to trigger neuronal cell death, which is a prominent feature of therapy-resistant temporal lobe epilepsy. Therefore, mitochondria have to be considered as promising targets for neuroprotective strategies in epilepsy.
authors.affiliation: Department of Epileptology, University Bonn Medical Center, Germany.
authors.forename: Alexei P
authors.initials: AP
authors.lastname: Kudin
authors.forename: Gábor
authors.initials: G
authors.lastname: Zsurka
authors.forename: Christian E
authors.initials: CE
authors.lastname: Elger
authors.forename: Wolfram S
authors.initials: WS
authors.lastname: Kunz
mesh_terms: D000255:Adenosine Triphosphate / Q000378:metabolism; D000818:Animals; D001921:Brain / Q000187:drug effects / Q000378:metabolism* / Q000473:pathology; D002118:Calcium / Q000378:metabolism; D016923:Cell Death; D004833:Epilepsy, Temporal Lobe / Q000209:etiology / Q000378:metabolism* / Q000473:pathology; D006801:Humans; D008928:Mitochondria / Q000378:metabolism* / Q000473:pathology; D009474:Neurons / Q000473:pathology; D018696:Neuroprotective Agents / Q000627:therapeutic use; D010766:Phosphorylation
chemical_list: D018696:Neuroprotective Agents; D000255:Adenosine Triphosphate; D002118:Calcium