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STATE-OF-THE-ART REVIEW ARTICLES: Richard H. Haas, Sumit Parikh, Marni J. Falk, Russell P. Saneto, Nicole I. Wolf, Niklas Darin, and Bruce H. Cohen Mitochondrial Disease: A Practical Approach for Primary Care Physicians Pediatrics 2007; 120: 1326-1333 [Abstract] [Full text] [PDF]

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Mitochondrial pathology

Heikki Savolainen   (4 December 2007)

Mitochondrial pathology 4 December 2007
Heikki Savolainen, Prof. Dept. of Occup. Safety & Hlth., Tampere, Finland Send letter to journal: Re: Mitochondrial pathology heikki.savolainen{at}stm.fi Heikki Savolainen	Besides the classical aspects of clinical pathology in mitochondrial energy metabolism so elegantly reviewed in this work, it may be interesting to regard the additional roles of succinate dehydrogenase (SDH) at the complex II of the respiratory chain. Its activity can be inhibited by toxicants, like malonic acid, 3-nitropropionic acid or alkoxyacetic acids (1) which leads to deranged electron flow in the respiratory chain. Accumulating succinate also inhibits the inactivation of hypoxia inducible factor by the proteasome. Acquired and inherited diminution of SDH has an important role e.g. in the epigenetic malignant transformation (2). Thus, mitochondria have central roles also in other acute and chronic diseases in addition to the classical neuromuscular syndromes. 1 Liesivuori J, Laitinen J, Savolainen H. 1999. Rat model for renal effects of 2-alkoxyalcohols and their acetates. Arch Toxicol 73: 229-232. 2 Baysal BE. A recurrent stop-codon mutation in succinate dehydrogenases B gene in normal peripheral blood and childhood T-cell acute leukemia. 2007. Plos ONE 2: e436. Conflict of Interest: None declared