The discovery of SDHD as a pheochromocytoma/paraganglioma susceptibility gene was the prismatic event that led to all of the subsequent work highlighting the key roles played by mitochondria in the pathogenesis of these tumors and other solid cancers. Alterations in the function of tricarboxylic acid cycle enzymes can cause accumulation of intermediate substrates and subsequent changes in cell metabolism, activation of the angiogenic pathway, increased reactive oxygen species production, DNA hypermethylation, and modification of the tumor microenvironment favoring tumor growth and aggressiveness. The elucidation of these tumorigenic mechanisms should lead to novel therapeutic targets for the treatment of the most aggressive forms of pheochromocytoma/paraganglioma.

15 YEARS OF PARAGANGLIOMA: Metabolism and pheochromocytoma/paraganglioma / Mannelli, Massimo; Rapizzi, Elena; Fucci, Rossella; Canu, Letizia; Ercolino, Tonino; Michaela, Luconi; Young William, F.. - In: ENDOCRINE-RELATED CANCER. - ISSN 1351-0088. - STAMPA. - 22:(2015), pp. 83-90. [10.1530/ERC-15-0215]

15 YEARS OF PARAGANGLIOMA: Metabolism and pheochromocytoma/paraganglioma

MANNELLI, MASSIMO;RAPIZZI, ELENA;FUCCI, ROSSELLA;CANU, LETIZIA;ERCOLINO, TONINO;LUCONI, MICHAELA;
2015

Abstract

The discovery of SDHD as a pheochromocytoma/paraganglioma susceptibility gene was the prismatic event that led to all of the subsequent work highlighting the key roles played by mitochondria in the pathogenesis of these tumors and other solid cancers. Alterations in the function of tricarboxylic acid cycle enzymes can cause accumulation of intermediate substrates and subsequent changes in cell metabolism, activation of the angiogenic pathway, increased reactive oxygen species production, DNA hypermethylation, and modification of the tumor microenvironment favoring tumor growth and aggressiveness. The elucidation of these tumorigenic mechanisms should lead to novel therapeutic targets for the treatment of the most aggressive forms of pheochromocytoma/paraganglioma.
2015
22
83
90
Mannelli, Massimo; Rapizzi, Elena; Fucci, Rossella; Canu, Letizia; Ercolino, Tonino; Michaela, Luconi; Young William, F.
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Utilizza questo identificatore per citare o creare un link a questa risorsa: https://hdl.handle.net/2158/1010923
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