Published in:
Open Access
01-12-2010 | Research article
Low aerobic mitochondrial energy metabolism in poorly- or undifferentiated neuroblastoma
Authors:
Rene' G Feichtinger, Franz Zimmermann, Johannes A Mayr, Daniel Neureiter, Cornelia Hauser-Kronberger, Freimut H Schilling, Neil Jones, Wolfgang Sperl, Barbara Kofler
Published in:
BMC Cancer
|
Issue 1/2010
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Abstract
Background
Succinate dehydrogenase (SDH) has been associated with carcinogenesis in pheochromocytoma and paraganglioma. In the present study we investigated components of the oxidative phosphorylation system in human neuroblastoma tissue samples.
Methods
Spectrophotometric measurements, immunohistochemical analysis and Western blot analysis were used to characterize the aerobic mitochondrial energy metabolism in neuroblastomas (NB).
Results
Compared to mitochondrial citrate synthase, SDH activity was severely reduced in NB (n = 14) versus kidney tissue. However no pathogenic mutations could be identified in any of the four subunits of SDH. Furthermore, no genetic alterations could be identified in the two novel SDH assembly factors SDHAF1 and SDH5. Alterations in genes encoding nfs-1, frataxin and isd-11 that could lead to a diminished SDH activity have not been detected in NB.
Conclusion
Because downregulation of other complexes of the oxidative phosphorylation system was also observed, a more generalized reduction of mitochondrial respiration seems to be present in neuroblastoma in contrast to the single enzyme defect found in hereditary pheochromocytomas.