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Am J Physiol Cell Physiol 291: C1114-C1120, 2006. First published June 7, 2006; doi:10.1152/ajpcell.00216.2006
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INVITED REVIEW

Tricarboxylic acid cycle dysfunction as a cause of human diseases and tumor formation

Jean-Jacques Brière,1 Judith Favier,2 Anne-Paule Gimenez-Roqueplo,2 and Pierre Rustin1

1Institut National de la Santé et de la Recherche Médicale (INSERM) Unité 676, Hôpital Robert Debré, Paris; and 2Département de Génétique, Hôpital Européen Georges Pompidou, Assistance Publique- Hôpitaux de Paris, Université Paris V and INSERM Unité 772, Collège de France, Paris, France

A renewed interest in tricarboxylic acid cycle enzymopathies has resulted from the report that, in addition to devastating encephalopathies, these can result in various types of tumors in human. We first review the major features of the cycle that may underlie this surprising variety of clinical features. After discussing the rare cases of encephalopathies associated with specific deficiencies of some of the tricarboxylic acid cycle enzyme, we finally examine the mechanism possibly causing tumor/cancer formation in the cases of mutations affecting fumarase or succinate dehydrogenase genes.

mitochondria; fumarase; succinate dehydrogenase; cancer; encephalopathy


Address for reprint requests and other correspondence: P. Rustin, INSERM U676, Hôpital Robert Debré, Batiment Ecran, 48, Boulevard Sérurier, 75019 Paris, France (e-mail: rustin{at}rdebre.inserm.fr)






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