Genetic Impairment of Succinate Metabolism Disrupts Bioenergetic Sensing in Adrenal Neuroendocrine Cancer

Gupta, Priyanka; Strange, Keehn; Telange, Rahul; Guo, Ailan; Hatch, Heather; Sobh, Amin; Elie, Jonathan; Carter, Angela M.; Totenhagen, John; Tan, Chunfeng; Sonawane, Yogesh A.; Neužil, Jiřı́; Natarajan, Amarnath; Ovens, Ashley J.; Oakhill, Jonathan S.; Wiederhold, Thorsten; Pacák, Karel; Ghayee, Hans K.; Meijer, Laurent; Reddy, Sushanth; Bibb, James A.

doi:10.2139/ssrn.4018612

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Isp2022 Highlights In Approaches and Models For Pgl Research1

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2023

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“…New and promising research models were presented at ISP2022, including two new Sdhbdeficient mouse strains (Gupta et al, 2022, Armstrong et al, 2022. Another emerging model of patient tumor-derived organoid cultures that may be amenable to drug screens may provide interesting information on therapeutic profile of PGLs (Dahia, Soragni et al, unpublished observations).…”

Section: Isp2022 Highlights In Approaches and Models For Pgl Researchmentioning

confidence: 99%

Update on the genetics of paragangliomas

Gimenez‐Roqueplo

Robledo

Dahia

2023

Endocrine-Related Cancer

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Paragangliomas (PGL) of the adrenal (also known as pheochromocytomas) or extra-adrenal neural crest-derived cells are highly heritable tumors, usually driven by single pathogenic variants that occur mutually exclusively in genes involved in multiple cellular processes, including the response to hypoxia, MAPK/ERK signaling and WNT signaling. Discovery of driver mutations has led to active clinical surveillance with outcome implications in familial PGL. The spectrum of mutations continues to grow and reveal unique mechanisms of tumorigenesis that inform tumor biology and provide the rationale for targeted therapy. Here we review recent progress in the genetics and molecular pathogenesis of PGLs and discuss new prospects for advancing research with new disease models and ongoing clinical trials presented at the recent International Symposium of Pheochromocytomas and Paragangliomas (ISP2022) held in October 2022 in Prague.

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Section: Isp2022 Highlights In Approaches and Models For Pgl Researchmentioning

confidence: 99%