Journal Article

Mitochondrial complex I is deficient in renal oncocytomas

Hélène Simonnet, Jocelyne Demont, Kathy Pfeiffer, Leïla Guenaneche, Raymonde Bouvier, Ulrich Brandt, Hermann Schägger and Catherine Godinot

in Carcinogenesis

Volume 24, issue 9, pages 1461-1466
Published in print September 2003 | ISSN: 0143-3334
Published online September 2003 | e-ISSN: 1460-2180 | DOI:
Mitochondrial complex I is deficient in renal oncocytomas

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Renal oncocytomas are benign tumors characterized by dense accumulation of mitochondria the cause of which remains unknown so far. Consistently, mitochondrial DNA content and the amounts and catalytic activities of several oxidative phosphorylation (OXPHOS) complexes were known to be increased in these tumors, but it was not ascertained that the OXPHOS system was functional. Here we investigated mitochondrial complex I and found that its NADH dehydrogenase activity and protein content were specifically decreased in oncocytomas, in stark contrast with the parallel decrease of all respiratory chain complexes in other, malignant, renal tumors. We conclude that deficiency of complex I in oncocytomas might be the early event causing the increased mitochondrial biogenesis, attempting to compensate for the loss of OXPHOS function. Since other tumors were found to be linked to mitochondrial deficiencies like genetic alterations of fumarate hydratase or succinate dehydrogenase, oncocytoma could be the third type of benign tumor associated with impairment of mitochondrial ATP production in an oxidative, quiescent tissue. Besides, complex I enzyme activity was moderately decreased in the vicinity of oncocytomas, when compared with normal tissue adjacent to other renal tumors. This suggested that oncocytomas are the result of at least two serial modifications altering the mitochondrial respiratory chain.

Keywords: CCRCCs, clear cell renal cell carcinomas; OXPHOS, oxidative phosphorylation; pVHL, von Hippel-Lindau tumor suppressor protein

Journal Article.  4027 words.  Illustrated.

Subjects: Clinical Cytogenetics and Molecular Genetics

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