Cytochrome c oxidase deficit is associated with the seizure onset zone in young patients with focal cortical dysplasia Type II

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• 作者：Lili Miles ; Hansel M. Greiner ; Francesco T. Mangano…
• 关键词：Cytochrome c oxidase ; Epilepsy surgery ; Focal cortical dysplasia ; Mitochondria ; Pathology
• 刊名：Metabolic Brain Disease
• 出版年：2015
• 出版时间：October 2015
• 年：2015
• 卷：30
• 期：5
• 页码：1151-1160
• 全文大小：1,298 KB
• 参考文献：Abdijadid S, Mathern GW, Levine MS, Cepeda C (2015) Basic mechanisms of epileptogenesis in pediatric cortical dysplasia. CNS Neurosci Ther?21:92-03
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• 作者单位：Lili Miles (1)
  Hansel M. Greiner (2)
  Francesco T. Mangano (3)
  Paul S. Horn (4)
  James L. Leach (5)
  Michael V. Miles (2)
  
  1. Division of Pathology and Laboratory Medicine, Cincinnati Children’s Hospital Medical Center, 3333 Burnet Ave., Cincinnati, OH, 45229, USA
  2. Division of Neurology, Department of Pediatrics, Cincinnati Children’s Hospital Medical, Cincinnati, OH, USA
  3. Division of Pediatric Neurosurgery, Department of Surgery, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA
  4. Divisions of Neurology and Biostatistics & Epidemiology, Department of Pediatrics, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA
  5. Department of Radiology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA
  
• 刊物类别：Biomedical and Life Sciences
• 刊物主题：Biomedicine
  Neurosciences
  Neurology
  Biochemistry
  Oncology
  
• 出版者：Springer Netherlands
• ISSN：1573-7365

文摘

It has been postulated that mitochondrial dysfunction may be an important factor in epileptogenesis of intractable epilepsy. The current study tests the hypothesis that mitochondrial Complex IV (CIV) or cytochrome c oxidase dysfunction is associated with the seizure onset zone (SOZ) in patients with focal cortical dysplasia (FCD). Subjects were selected based on: age <19y; epilepsy surgery between May, 2010 and October, 2011; pathological diagnosis of isolated focal cortical dysplasia Type I (FCDI) or Type II (FCDII); and sufficient residual cortical tissue to conduct analysis of electron transport chain complex (ETC) activity in SOZ and adjacent cortical regions. In this retrospective study, patients were identified who had sufficient unfixed, frozen brain tissue for biochemical analysis in tissue homogenates. Specimens were subtyped using ILAE classification for FCD, and excluded if diagnosed with FCD Type III or dual pathology. Analysis of ETC activity in resected tissues was conducted independently and without knowledge of the identity, diagnosis, or clinical status of individual subjects. Seventeen patients met the inclusion criteria, including 6 FCDI and 11 FCDII. Comparison of adjacent cortical resections showed decreased CIV activity in the SOZ of the FCDII group (P--.003), but no significant CIV difference in adjacent tissues of the FCDI group. Because of the importance of CIV as the terminal and rate-limiting complex in the mitochondrial electron transport chain, these authors conclude that 1) a deficit of CIV is associated with the SOZ of patients with FCDII; 2) CIV deficiency may contribute to the spectrum of FCD neuropathology; and 3) further investigation of CIV in FCD may lead to the discovery of new targets for neuroprotective therapies for patients with intractable epilepsy. Keywords Cytochrome c oxidase Epilepsy surgery Focal cortical dysplasia Mitochondria Pathology