Epigenetics of pheochromocytoma and paraganglioma

MOLECULAR AND CELLULAR ENDOCRINOLOGY

卷:469

Article

Bjorklund, Peyman¹  Backman, Samuel¹ 

[1] Uppsala Univ, Dept Surg Sci, Expt Surg, Uppsala, Sweden

关键词: Pheochromocytoma;    Paraganglioma;    Epigenetics;    Adrenal;    Methylation;   

DOI  :  10.1016/j.mce.2017.06.016

来源: Elsevier

【 摘 要 】

Pheochromocytomas and paragangliomas (PPGLs) are neuroendocrine tumors arising in the medullae of the adrenal glands or in paraganglia. The knowledge of the tumor biology of these lesions has increased dramatically during the past two decades and more than a dozen recurrently mutated genes have been identified. Different clusters have been described that share epigenetic signatures. Mutations in the succinate dehydrogenase complex subunit genes play a pivotal role in reprogramming the epigenetic state of these tumors by inhibiting epigenetic regulators such as TET enzymes and histone demethylases. Another subgroup of tumors carries hypomethylated genomes, and overexpression of several microRNAs has been described. While much remains to be investigated regarding the epigenetics of PPGLs, it is clear that it plays an important role in PPGL biology. (C) 2017 Elsevier B.V. All rights reserved.

【 授权许可】

Free