Kunz 2000 Ann Neurol

From Bioblast
Publications in the MiPMap
Kunz WS, Kudin AP, Vielhaber S, Blumecke I, Zuschratter W, Schramm J, Beck H, Elger CE (2000) Mitochondrial complex I deficiency in the epileptic focus of patients with temporal lobe epilepsy. Ann Neurol 48:766-73.

Β» PMID: 11079540

Kunz WS, Kudin AP, Vielhaber S, Blumecke I, Zuschratter W, Schramm J, Beck H, Elger CE (2000) Ann Neurol

Abstract: Mitochondria are cellular organelles crucial for energy supply and calcium homeostasis in neuronal cells, and their dysfunction causes seizure activity in some rare human epilepsies. To directly test whether mitochondrial respiratory chain enzymes are abnormal in the most common form of chronic epilepsy, temporal lobe epilepsy (TLE), living human brain specimens from 57 epileptic patients and 2 nonepileptic controls were investigated. In TLE patients with a hippocampal epileptic focus, we demonstrated a specific deficiency of complex I of the mitochondrial respiratory chain in the hippocampal CA3 region. In contrast, TLE patients with a parahippocampal epileptic focus showed reduced complex I activity only in parahippocampal tissue. Inhibitor titrations of the maximal respiration rate of intact human brain slices revealed that the observed reduction in complex I activity is sufficient to affect the adenosine triphosphate production rate. The abnormal complex I activity in the hippocampal CA3 region was paralleled by increased succinate dehydrogenase staining of neurons and marked ultrastructural abnormalities of mitochondria. Therefore, mitochondrial dysfunction is suggested to be specific for the epileptic focus and may constitute a pathomechanism contributing to altered excitability and selective neuronal vulnerability in TLE.


Stress:Mitochondrial disease  Organism: Human  Tissue;cell: Nervous system 

Enzyme: Complex I, TCA cycle and matrix dehydrogenases 

Coupling state: OXPHOS 

HRR: Oxygraph-2k 

Cookies help us deliver our services. By using our services, you agree to our use of cookies.