Article ; Online: MITOCHONDRIA: Succinate dehydrogenase subunit B-associated phaeochromocytoma and paraganglioma.
The international journal of biochemistry & cell biology
2021 Volume 134, Page(s) 105949
Abstract: Phaeochromocytomas and paragangliomas are rare neuroendocrine tumours. So far, over 20 causative genes have been identified, of which the most frequent and strongest indicator for malignancies are mutations in succinate dehydrogenase subunit B. No ... ...
| Abstract | Phaeochromocytomas and paragangliomas are rare neuroendocrine tumours. So far, over 20 causative genes have been identified, of which the most frequent and strongest indicator for malignancies are mutations in succinate dehydrogenase subunit B. No curative therapy is available for patients with metastases resulting in poor prognosis. Therapy development has been hindered by lack of suitable model systems. The succinate dehydrogenase complex is located in the inner membrane of the mitochondria and plays a crucial role in the oxidative phosphorylation chain and the tricarboxylic acid-cycle. Succinate dehydrogenase deficiency results in accumulation of the oncometabolite succinate inducing hypoxia inducible factor stabilization, deoxyribonucleic acid and histone methylation inhibition, and impaired production of adenosine triphosphate. It remains unknown which combination of pathways and/or triggers are decisive for metastases development. In this review, the role of mitochondria in malignant succinate dehydrogenase subunit B-associated phaeochromocytomas and paragangliomas and implications for mitochondria as therapeutic target are discussed. |
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| MeSH term(s) | Adrenal Gland Neoplasms/enzymology ; Adrenal Gland Neoplasms/genetics ; Adrenal Gland Neoplasms/metabolism ; Adrenal Gland Neoplasms/pathology ; Animals ; Electron Transport Complex II/deficiency ; Electron Transport Complex II/genetics ; Electron Transport Complex II/metabolism ; Humans ; Hypoxia-Inducible Factor 1, alpha Subunit/metabolism ; Metabolism, Inborn Errors/enzymology ; Metabolism, Inborn Errors/genetics ; Metabolism, Inborn Errors/metabolism ; Metabolism, Inborn Errors/pathology ; Mitochondria/enzymology ; Mitochondria/genetics ; Mitochondria/metabolism ; Mitochondria/pathology ; Mitochondrial Diseases/enzymology ; Mitochondrial Diseases/genetics ; Mitochondrial Diseases/metabolism ; Mitochondrial Diseases/pathology ; Mutation ; Paraganglioma/enzymology ; Paraganglioma/genetics ; Paraganglioma/metabolism ; Paraganglioma/pathology ; Pheochromocytoma/enzymology ; Pheochromocytoma/genetics ; Pheochromocytoma/metabolism ; Pheochromocytoma/pathology ; Reactive Oxygen Species/metabolism ; Succinate Dehydrogenase/genetics ; Succinate Dehydrogenase/metabolism |
| Chemical Substances | HIF1A protein, human ; Hypoxia-Inducible Factor 1, alpha Subunit ; Reactive Oxygen Species ; Electron Transport Complex II (EC 1.3.5.1) ; SDHB protein, human (EC 1.3.5.1) ; Succinate Dehydrogenase (EC 1.3.99.1) |
| Language | English |
| Publishing date | 2021-02-17 |
| Publishing country | Netherlands |
| Document type | Journal Article ; Research Support, Non-U.S. Gov't ; Review |
| ZDB-ID | 1228429-4 |
| ISSN | 1878-5875 ; 1357-2725 |
| ISSN (online) | 1878-5875 |
| ISSN | 1357-2725 |
| DOI | 10.1016/j.biocel.2021.105949 |
| Database | MEDical Literature Analysis and Retrieval System OnLINE |
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