Journal article

A xenograft and cell line model of SDH-deficient pheochromocytoma derived from Sdhb plus /- rats

James F Powers, Brent Cochran, James D Baleja, Hadley D Sikes, Andrew D Pattison, Xue Zhang, Inna Lomakin, Annette Shepard-Barry, Karel Pacak, Sun Jin Moon, Troy F Langford, Kassi Taylor Stein, Richard W Tothill, Yingbin Ouyang, Arthur S Tischler

Endocrine-Related Cancer | BIOSCIENTIFICA LTD | Published : 2020


Tumors caused by loss-of-function mutations in genes encoding TCA cycle enzymes have been recently discovered and are now of great interest. Mutations in succinate dehydrogenase (SDH) subunits cause pheochromocytoma/paraganglioma (PCPG) and syndromically associated tumors, which differ phenotypically and clinically from more common SDH-intact tumors of the same types. Consequences of SDH deficiency include rewired metabolism, pseudohypoxic signaling and altered redox balance. PCPG with SDHB mutations are particularly aggressive, and development of treatments has been hampered by lack of valid experimental models. Attempts to develop mouse models have been unsuccessful. Using a new strategy, ..

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Awarded by National Institutes of Health SIG grant

Funding Acknowledgements

This research was supported principally by grants to A S T from the SDHB Pheo Para Coalition, the Pheo Para Alliance and the Paradifference Foundation and by the Intramural Research Program of the Eunice Kennedy Shriver NICHD, NIH. The work utilized NMR instrumentation purchased from a National Institutes of Health SIG grant (S10OD020073). A D P is supported by the Joseph Herman Trust. R W T is supported by a fellowship from the Victorian Cancer Agency.