IMR Press / FBE / Volume 3 / Issue 1 / DOI: 10.2741/E231

Frontiers in Bioscience-Elite (FBE) is published by IMR Press from Volume 13 Issue 2 (2021). Previous articles were published by another publisher on a subscription basis, and they are hosted by IMR Press on as a courtesy and upon agreement with Frontiers in Bioscience.

Loss of mitochondria in ganglioneuromas
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1 Department of Pediatrics, University Hospital Salzburg, Paracelsus Medical University, 5020- Salzburg, Austria
2 Department of Pathology, University Hospital Salzburg, Paracelsus Medical University, 5020-Salzburg, Austria
3 Children’s Hospital, Department of Pediatric Oncology and Hematology, University of Cologne, 50924- Cologne, Germany

*Author to whom correspondence should be addressed.

Academic Editor: Anna Czarnecka

Front. Biosci. (Elite Ed) 2011, 3(1), 179–186;
Published: 1 January 2011
(This article belongs to the Special Issue Frontiers in molecular medicine)

A shift in cellular energy production from oxidative phosphorylation (OXPHOS) to glycolysis, even under aerobic conditions, is called the Warburg effect. To elucidate changes of the mitochondrial energy metabolism in ganglioneuroma (GN) individual OXPHOS enzymes were analyzed by activity assays and by immunohistochemical staining methods. GN (n=7) showed a significant reduction in the activity and content of OXPHOS enzymes. Citrate synthase activity was also severely diminished in GN compared to normal cortical kidney (p=0.0002) and adrenal (p=0.0024) tissues. Furthermore, the mitochondrial membrane protein porin was undetectable or significantly reduced. Accordingly, a reduction of the copy number of mitochondrial DNA was observed in GN compared to cortical kidney tissue. The striking decline of mitochondrial mass is specific for GN but not for neuroblastoma, in which a reduction of the OXPHOS complexes without reduction of mitochondrial mass was reported. Knowledge of the mechanism by which tumor cells achieve the Warburg effect will provide a starting point for functional studies aimed at restoring aerobic energy metabolism as a potential new therapeutic strategy to treat malignancies.

Warburg effect
Respiratory Chain Enzyme Deficiency
Mitochondrial Mass
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