{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,19]],"date-time":"2026-10-19T23:18:30Z","timestamp":1792451910243,"version":"4.5.0"},"reference-count":142,"publisher":"Springer Science and Business Media LLC","issue":"4","license":[{"start":{"date-parts":[[2021,12,1]],"date-time":"2021-12-01T00:00:00Z","timestamp":1638316800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2021,12,27]],"date-time":"2021-12-27T00:00:00Z","timestamp":1640563200000},"content-version":"vor","delay-in-days":26,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100003825","name":"Magyar Tudom\u00e1nyos Akad\u00e9mia","doi-asserted-by":"publisher","award":["OTKA K134285"],"award-info":[{"award-number":["OTKA K134285"]}],"id":[{"id":"10.13039\/501100003825","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100009934","name":"E\u00f6tv\u00f6s Lor\u00e1nd University","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100009934","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Cancer Metastasis Rev"],"published-print":{"date-parts":[[2021,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Pheochromocytoma (PHEO) and paraganglioma (PGL) (together PPGL) are tumors with poor outcomes that arise from neuroendocrine cells in the adrenal gland, and sympathetic and parasympathetic ganglia outside the adrenal gland, respectively. Many follow germline mutations in genes coding for subunits of succinate dehydrogenase (SDH), a tetrameric enzyme in the tricarboxylic acid (TCA) cycle that both converts succinate to fumarate and participates in electron transport. Germline<jats:italic>SDH<\/jats:italic>subunit<jats:italic>B<\/jats:italic>(<jats:italic>SDHB<\/jats:italic>) mutations have a high metastatic potential. Herein, we review the spectrum of model organisms that have contributed hugely to our understanding of SDH dysfunction. In<jats:italic>Saccharomyces cerevisiae<\/jats:italic>(yeast), succinate accumulation inhibits alpha-ketoglutarate-dependent dioxygenase enzymes leading to DNA demethylation. In the worm<jats:italic>Caenorhabditis elegans<\/jats:italic>, mutated SDH creates developmental abnormalities, metabolic rewiring, an energy deficit and oxygen hypersensitivity (the latter is also found in<jats:italic>Drosophila melanogaster<\/jats:italic>). In the zebrafish<jats:italic>Danio rerio<\/jats:italic>,<jats:italic>sdhb<\/jats:italic>mutants display a shorter lifespan with defective energy metabolism. Recently,<jats:italic>SDHB<\/jats:italic>-deficient pheochromocytoma has been cultivated in xenografts and has generated cell lines, which can be traced back to a heterozygous<jats:italic>SDHB<\/jats:italic>-deficient rat. We propose that a combination of such models can be efficiently and effectively used in both pathophysiological studies and drug-screening projects in order to find novel strategies in PPGL treatment.<\/jats:p>","DOI":"10.1007\/s10555-021-10009-z","type":"journal-article","created":{"date-parts":[[2021,12,27]],"date-time":"2021-12-27T00:02:40Z","timestamp":1640563360000},"page":"1177-1201","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["Model systems in SDHx-related pheochromocytoma\/paraganglioma"],"prefix":"10.1007","volume":"40","author":[{"given":"Krisztina","family":"Tak\u00e1cs-Vellai","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zsolt","family":"Farkas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fanni","family":"\u0150sz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gordon W.","family":"Stewart","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,12,27]]},"reference":[{"issue":"6","key":"10009_CR1","doi-asserted-by":"publisher","first-page":"552","DOI":"10.1056\/NEJMra1806651","volume":"381","author":"HPH Neumann","year":"2019","unstructured":"Neumann, H. P. H., Young, W. F., Jr., & Eng, C. (2019). Pheochromocytoma and paraganglioma. New England Journal of Medicine, 381(6), 552\u2013565. https:\/\/doi.org\/10.1056\/NEJMra1806651","journal-title":"New England Journal of Medicine"},{"issue":"4","key":"10009_CR2","doi-asserted-by":"publisher","first-page":"393","DOI":"10.1016\/j.mito.2010.03.001","volume":"10","author":"J Rutter","year":"2010","unstructured":"Rutter, J., Winge, D. R., & Schiffman, J. D. (2010). Succinate dehydrogenase-Assembly, regulation and role in human disease. Mitochondrion, 10(4), 393\u2013401. https:\/\/doi.org\/10.1016\/j.mito.2010.03.001","journal-title":"Mitochondrion"},{"issue":"5","key":"10009_CR3","doi-asserted-by":"publisher","first-page":"289","DOI":"10.1038\/sj.ejhg.5200793","volume":"10","author":"P Rustin","year":"2002","unstructured":"Rustin, P., Munnich, A., & Rotig, A. (2002). Succinate dehydrogenase and human diseases: New insights into a well-known enzyme. European Journal of Human Genetics, 10(5), 289\u2013291. https:\/\/doi.org\/10.1038\/sj.ejhg.5200793","journal-title":"European Journal of Human Genetics"},{"issue":"2","key":"10009_CR4","doi-asserted-by":"publisher","first-page":"168","DOI":"10.3109\/10409238.2014.990556","volume":"50","author":"JG Van Vranken","year":"2015","unstructured":"Van Vranken, J. G., Na, U., Winge, D. R., & Rutter, J. (2015). Protein-mediated assembly of succinate dehydrogenase and its cofactors. Critical Reviews in Biochemistry and Molecular Biology, 50(2), 168\u2013180. https:\/\/doi.org\/10.3109\/10409238.2014.990556","journal-title":"Critical Reviews in Biochemistry and Molecular Biology"},{"issue":"2","key":"10009_CR5","doi-asserted-by":"publisher","first-page":"171","DOI":"10.1007\/s11845-008-0148-9","volume":"177","author":"C Cronin","year":"2008","unstructured":"Cronin, C. (2008). Charles Sugrue, M.D., of Cork (1775-1816) and the first description of a classical medical condition: Phaeochromocytoma. Irish Journal of Medical Science, 177(2), 171\u2013175. https:\/\/doi.org\/10.1007\/s11845-008-0148-9","journal-title":"Irish Journal of Medical Science"},{"issue":"7","key":"10009_CR6","doi-asserted-by":"publisher","first-page":"594","DOI":"10.1002\/bjs.1800740717","volume":"74","author":"RB Welbourn","year":"1987","unstructured":"Welbourn, R. B. (1987). Early surgical history of phaeochromocytoma. British Journal of Surgery, 74(7), 594\u2013596. https:\/\/doi.org\/10.1002\/bjs.1800740717","journal-title":"British Journal of Surgery"},{"key":"10009_CR7","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1196\/annals.1353.001","volume":"1073","author":"WM Manger","year":"2006","unstructured":"Manger, W. M. (2006). An overview of pheochromocytoma: History, current concepts, vagaries, and diagnostic challenges. Annals of the New York Academy of Sciences, 1073, 1\u201320. https:\/\/doi.org\/10.1196\/annals.1353.001","journal-title":"Annals of the New York Academy of Sciences"},{"issue":"3","key":"10009_CR8","doi-asserted-by":"publisher","first-page":"273","DOI":"10.3390\/cancers11030273","volume":"11","author":"LV Lotti","year":"2019","unstructured":"Lotti, L. V., Vespa, S., Pantalone, M. R., Perconti, S., Esposito, D. L., Visone, R., et al. (2019). A developmental perspective on paragangliar tumorigenesis. Cancers (Basel), 11(3), 273. https:\/\/doi.org\/10.3390\/cancers11030273","journal-title":"Cancers (Basel)"},{"issue":"5","key":"10009_CR9","doi-asserted-by":"publisher","first-page":"779","DOI":"10.1007\/s00401-017-1799-2","volume":"135","author":"F Verginelli","year":"2018","unstructured":"Verginelli, F., Perconti, S., Vespa, S., Schiavi, F., Prasad, S. C., Lanuti, P., et al. (2018). Paragangliomas arise through an autonomous vasculo-angio-neurogenic program inhibited by imatinib. Acta Neuropathologica, 135(5), 779\u2013798. https:\/\/doi.org\/10.1007\/s00401-017-1799-2","journal-title":"Acta Neuropathologica"},{"key":"10009_CR10","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1002\/path.1700360102","volume":"36","author":"WH Chase","year":"1933","unstructured":"Chase, W. H. (1933). Familial and bilateral tumours of the carotid body. Journal of Pathology and Bacteriology, 36, 1\u201312. https:\/\/doi.org\/10.1002\/path.1700360102","journal-title":"Journal of Pathology and Bacteriology"},{"issue":"5454","key":"10009_CR11","doi-asserted-by":"publisher","first-page":"848","DOI":"10.1126\/science.287.5454.848","volume":"287","author":"BE Baysal","year":"2000","unstructured":"Baysal, B. E., Ferrell, R. E., Willett-Brozick, J. E., Lawrence, E. C., Myssiorek, D., Bosch, A., et al. (2000). Mutations in SDHD, a mitochondrial complex II gene, in hereditary paraganglioma. Science, 287(5454), 848\u2013851. https:\/\/doi.org\/10.1126\/science.287.5454.848","journal-title":"Science"},{"issue":"3","key":"10009_CR12","doi-asserted-by":"publisher","first-page":"268","DOI":"10.1038\/81551","volume":"26","author":"S Niemann","year":"2000","unstructured":"Niemann, S., & Muller, U. (2000). Mutations in SDHC cause autosomal dominant paraganglioma, type 3. Nature Genetics, 26(3), 268\u2013270. https:\/\/doi.org\/10.1038\/81551","journal-title":"Nature Genetics"},{"issue":"1","key":"10009_CR13","doi-asserted-by":"publisher","first-page":"49","DOI":"10.1086\/321282","volume":"69","author":"D Astuti","year":"2001","unstructured":"Astuti, D., Latif, F., Dallol, A., Dahia, P. L., Douglas, F., George, E., et al. (2001). Gene mutations in the succinate dehydrogenase subunit SDHB cause susceptibility to familial pheochromocytoma and to familial paraganglioma. American Journal of Human Genetics, 69(1), 49\u201354. https:\/\/doi.org\/10.1086\/321282","journal-title":"American Journal of Human Genetics"},{"issue":"5944","key":"10009_CR14","doi-asserted-by":"publisher","first-page":"1139","DOI":"10.1126\/science.1175689","volume":"325","author":"HX Hao","year":"2009","unstructured":"Hao, H. X., Khalimonchuk, O., Schraders, M., Dephoure, N., Bayley, J. P., Kunst, H., et al. (2009). SDH5, a gene required for flavination of succinate dehydrogenase, is mutated in paraganglioma. Science, 325(5944), 1139\u20131142. https:\/\/doi.org\/10.1126\/science.1175689","journal-title":"Science"},{"key":"10009_CR15","doi-asserted-by":"publisher","DOI":"10.1093\/jnci\/djab158","author":"K Hadrava Vanova","year":"2021","unstructured":"Hadrava Vanova, K., Pang, Y., Krobova, L., Kraus, M., Nahacka, Z., Boukalova, S., et al. (2021). Germline SUCLG2 variants in patients with pheochromocytoma and paraganglioma. Journal of the National Cancer Institute. https:\/\/doi.org\/10.1093\/jnci\/djab158","journal-title":"Journal of the National Cancer Institute"},{"issue":"11","key":"10009_CR16","doi-asserted-by":"publisher","first-page":"1432","DOI":"10.1016\/j.bbabio.2011.07.003","volume":"1807","author":"C Bardella","year":"2011","unstructured":"Bardella, C., Pollard, P. J., & Tomlinson, I. (2011). SDH mutations in cancer. Biochimica et Biophysica Acta, 1807(11), 1432\u20131443. https:\/\/doi.org\/10.1016\/j.bbabio.2011.07.003","journal-title":"Biochimica et Biophysica Acta"},{"issue":"34","key":"10009_CR17","doi-asserted-by":"publisher","first-page":"8812","DOI":"10.1200\/JCO.2005.03.1484","volume":"23","author":"L Amar","year":"2005","unstructured":"Amar, L., Bertherat, J., Baudin, E., Ajzenberg, C., Bressac-de Paillerets, B., Chabre, O., et al. (2005). Genetic testing in pheochromocytoma or functional paraganglioma. Journal of Clinical Oncology, 23(34), 8812\u20138818. https:\/\/doi.org\/10.1200\/JCO.2005.03.1484","journal-title":"Journal of Clinical Oncology"},{"issue":"3","key":"10009_CR18","doi-asserted-by":"publisher","first-page":"779","DOI":"10.1210\/jc.2006-2315","volume":"92","author":"HJ Timmers","year":"2007","unstructured":"Timmers, H. J., Kozupa, A., Eisenhofer, G., Raygada, M., Adams, K. T., Solis, D., et al. (2007). Clinical presentations, biochemical phenotypes, and genotype-phenotype correlations in patients with succinate dehydrogenase subunit B-associated pheochromocytomas and paragangliomas. Journal of Clinical Endocrinology and Metabolism, 92(3), 779\u2013786. https:\/\/doi.org\/10.1210\/jc.2006-2315","journal-title":"Journal of Clinical Endocrinology and Metabolism"},{"issue":"7","key":"10009_CR19","doi-asserted-by":"publisher","first-page":"L43","DOI":"10.1530\/ERC-17-0030","volume":"24","author":"N Tufton","year":"2017","unstructured":"Tufton, N., Ghelani, R., Srirangalingam, U., Kumar, A. V., Drake, W. M., Iacovazzo, D., et al. (2017). SDHA mutated paragangliomas may be at high risk of metastasis. Endocrine-Related Cancer, 24(7), L43\u2013L49. https:\/\/doi.org\/10.1530\/ERC-17-0030","journal-title":"Endocrine-Related Cancer"},{"issue":"1","key":"10009_CR20","doi-asserted-by":"publisher","first-page":"314","DOI":"10.1073\/pnas.1009199108","volume":"108","author":"KA Janeway","year":"2011","unstructured":"Janeway, K. A., Kim, S. Y., Lodish, M., Nose, V., Rustin, P., Gaal, J., et al. (2011). Defects in succinate dehydrogenase in gastrointestinal stromal tumors lacking KIT and PDGFRA mutations. Proceedings of the National Academy of Sciences, 108(1), 314\u2013318. https:\/\/doi.org\/10.1073\/pnas.1009199108","journal-title":"Proceedings of the National Academy of Sciences"},{"issue":"17","key":"10009_CR21","doi-asserted-by":"publisher","first-page":"1260","DOI":"10.1093\/jnci\/djn254","volume":"100","author":"C Ricketts","year":"2008","unstructured":"Ricketts, C., Woodward, E. R., Killick, P., Morris, M. R., Astuti, D., Latif, F., et al. (2008). Germline SDHB mutations and familial renal cell carcinoma. Journal of the National Cancer Institute, 100(17), 1260\u20131262. https:\/\/doi.org\/10.1093\/jnci\/djn254","journal-title":"Journal of the National Cancer Institute"},{"issue":"2","key":"10009_CR22","doi-asserted-by":"publisher","first-page":"121","DOI":"10.1530\/ERC-14-0537","volume":"22","author":"Y Ni","year":"2015","unstructured":"Ni, Y., Seballos, S., Ganapathi, S., Gurin, D., Fletcher, B., Ngeow, J., et al. (2015). Germline and somatic SDHx alterations in apparently sporadic differentiated thyroid cancer. Endocrine-Related Cancer, 22(2), 121\u2013130. https:\/\/doi.org\/10.1530\/ERC-14-0537","journal-title":"Endocrine-Related Cancer"},{"issue":"1","key":"10009_CR23","doi-asserted-by":"publisher","first-page":"6","DOI":"10.1016\/j.trecan.2017.11.001","volume":"4","author":"I Jochmanova","year":"2018","unstructured":"Jochmanova, I., & Pacak, K. (2018). Genomic landscape of pheochromocytoma and paraganglioma. Trends Cancer, 4(1), 6\u20139. https:\/\/doi.org\/10.1016\/j.trecan.2017.11.001","journal-title":"Trends Cancer"},{"key":"10009_CR24","doi-asserted-by":"publisher","DOI":"10.1210\/endrev\/bnab019","author":"S Nolting","year":"2021","unstructured":"Nolting, S., Bechmann, N., Taieb, D., Beuschlein, F., Fassnacht, M., Kroiss, M., et al. (2021). Personalized management of pheochromocytoma and paraganglioma. Endocrine Reviews. https:\/\/doi.org\/10.1210\/endrev\/bnab019","journal-title":"Endocrine Reviews"},{"issue":"8","key":"10009_CR25","doi-asserted-by":"publisher","first-page":"T201","DOI":"10.1530\/ERC-18-0085","volume":"25","author":"HP Neumann","year":"2018","unstructured":"Neumann, H. P., Young, W. F., Jr., Krauss, T., Bayley, J. P., Schiavi, F., Opocher, G., et al. (2018). 65 years OF the double helix: Genetics informs precision practice in the diagnosis and management of pheochromocytoma. Endocrine-Related Cancer, 25(8), T201\u2013T219. https:\/\/doi.org\/10.1530\/ERC-18-0085","journal-title":"Endocrine-Related Cancer"},{"issue":"6","key":"10009_CR26","doi-asserted-by":"publisher","first-page":"489","DOI":"10.1210\/er.2017-00062","volume":"38","author":"J Crona","year":"2017","unstructured":"Crona, J., Taieb, D., & Pacak, K. (2017). New perspectives on pheochromocytoma and paraganglioma: Toward a molecular classification. Endocrine Reviews, 38(6), 489\u2013515. https:\/\/doi.org\/10.1210\/er.2017-00062","journal-title":"Endocrine Reviews"},{"issue":"2","key":"10009_CR27","doi-asserted-by":"publisher","first-page":"82","DOI":"10.1016\/j.cancergencyto.2009.04.009","volume":"192","author":"P Prasad","year":"2009","unstructured":"Prasad, P., Kant, J. A., Wills, M., O\u2019Leary, M., Lovvorn, H., 3rd., & Yang, E. (2009). Loss of heterozygosity of succinate dehydrogenase B mutation by direct sequencing in synchronous paragangliomas. Cancer Genetics and Cytogenetics, 192(2), 82\u201385. https:\/\/doi.org\/10.1016\/j.cancergencyto.2009.04.009","journal-title":"Cancer Genetics and Cytogenetics"},{"issue":"8","key":"10009_CR28","doi-asserted-by":"publisher","first-page":"764","DOI":"10.1016\/S1470-2045(09)70164-0","volume":"10","author":"FH van Nederveen","year":"2009","unstructured":"van Nederveen, F. H., Gaal, J., Favier, J., Korpershoek, E., Oldenburg, R. A., de Bruyn, E. M., et al. (2009). An immunohistochemical procedure to detect patients with paraganglioma and phaeochromocytoma with germline SDHB, SDHC, or SDHD gene mutations: A retrospective and prospective analysis. The Lancet Oncology, 10(8), 764\u2013771. https:\/\/doi.org\/10.1016\/S1470-2045(09)70164-0","journal-title":"The Lancet Oncology"},{"issue":"5","key":"10009_CR29","doi-asserted-by":"publisher","first-page":"1088","DOI":"10.1016\/j.bpj.2016.07.028","volume":"111","author":"Z Dai","year":"2016","unstructured":"Dai, Z., Shestov, A. A., Lai, L., & Locasale, J. W. (2016). A flux balance of glucose metabolism clarifies the requirements of the Warburg effect. Biophysical Journal, 111(5), 1088\u20131100. https:\/\/doi.org\/10.1016\/j.bpj.2016.07.028","journal-title":"Biophysical Journal"},{"key":"10009_CR30","doi-asserted-by":"publisher","first-page":"132","DOI":"10.1016\/j.canlet.2019.03.020","volume":"452","author":"S Saikolappan","year":"2019","unstructured":"Saikolappan, S., Kumar, B., Shishodia, G., Koul, S., & Koul, H. K. (2019). Reactive oxygen species and cancer: A complex interaction. Cancer Letters, 452, 132\u2013143. https:\/\/doi.org\/10.1016\/j.canlet.2019.03.020","journal-title":"Cancer Letters"},{"issue":"1","key":"10009_CR31","doi-asserted-by":"publisher","first-page":"77","DOI":"10.1016\/j.ccr.2004.11.022","volume":"7","author":"MA Selak","year":"2005","unstructured":"Selak, M. A., Armour, S. M., MacKenzie, E. D., Boulahbel, H., Watson, D. G., Mansfield, K. D., et al. (2005). Succinate links TCA cycle dysfunction to oncogenesis by inhibiting HIF-alpha prolyl hydroxylase. Cancer Cell, 7(1), 77\u201385. https:\/\/doi.org\/10.1016\/j.ccr.2004.11.022","journal-title":"Cancer Cell"},{"issue":"12","key":"10009_CR32","doi-asserted-by":"publisher","first-page":"710","DOI":"10.1038\/s41568-020-00303-3","volume":"20","author":"JA Losman","year":"2020","unstructured":"Losman, J. A., Koivunen, P., & Kaelin, W. G., Jr. (2020). 2-Oxoglutarate-dependent dioxygenases in cancer. Nature Reviews Cancer, 20(12), 710\u2013726. https:\/\/doi.org\/10.1038\/s41568-020-00303-3","journal-title":"Nature Reviews Cancer"},{"issue":"12","key":"10009_CR33","doi-asserted-by":"publisher","first-page":"1326","DOI":"10.1101\/gad.191056.112","volume":"26","author":"M Xiao","year":"2012","unstructured":"Xiao, M., Yang, H., Xu, W., Ma, S., Lin, H., Zhu, H., et al. (2012). Inhibition of alpha-KG-dependent histone and DNA demethylases by fumarate and succinate that are accumulated in mutations of FH and SDH tumor suppressors. Genes & Development, 26(12), 1326\u20131338. https:\/\/doi.org\/10.1101\/gad.191056.112","journal-title":"Genes & Development"},{"key":"10009_CR34","unstructured":"Mikhail, M. I., & Singh, A. K. (2021). Von Hippel Lindau syndrome. In StatPearls. Treasure Island (FL)."},{"issue":"7","key":"10009_CR35","doi-asserted-by":"publisher","first-page":"517","DOI":"10.1016\/j.tibs.2018.04.002","volume":"43","author":"CQ Herr","year":"2018","unstructured":"Herr, C. Q., & Hausinger, R. P. (2018). Amazing diversity in biochemical roles of Fe(II)\/2-oxoglutarate oxygenases. Trends in Biochemical Sciences, 43(7), 517\u2013532. https:\/\/doi.org\/10.1016\/j.tibs.2018.04.002","journal-title":"Trends in Biochemical Sciences"},{"issue":"6","key":"10009_CR36","doi-asserted-by":"publisher","first-page":"739","DOI":"10.1016\/j.ccr.2013.04.018","volume":"23","author":"E Letouze","year":"2013","unstructured":"Letouze, E., Martinelli, C., Loriot, C., Burnichon, N., Abermil, N., Ottolenghi, C., et al. (2013). SDH mutations establish a hypermethylator phenotype in paraganglioma. Cancer Cell, 23(6), 739\u2013752. https:\/\/doi.org\/10.1016\/j.ccr.2013.04.018","journal-title":"Cancer Cell"},{"issue":"24","key":"10009_CR37","doi-asserted-by":"publisher","first-page":"3136","DOI":"10.1093\/hmg\/ddm275","volume":"16","author":"EH Smith","year":"2007","unstructured":"Smith, E. H., Janknecht, R., & Maher, L. J., 3rd. (2007). Succinate inhibition of alpha-ketoglutarate-dependent enzymes in a yeast model of paraganglioma. Human Molecular Genetics, 16(24), 3136\u20133148. https:\/\/doi.org\/10.1093\/hmg\/ddm275","journal-title":"Human Molecular Genetics"},{"issue":"2","key":"10009_CR38","doi-asserted-by":"publisher","first-page":"155","DOI":"10.1016\/j.ccr.2005.06.015","volume":"8","author":"S Lee","year":"2005","unstructured":"Lee, S., Nakamura, E., Yang, H., Wei, W., Linggi, M. S., Sajan, M. P., et al. (2005). Neuronal apoptosis linked to EglN3 prolyl hydroxylase and familial pheochromocytoma genes: Developmental culling and cancer. Cancer Cell, 8(2), 155\u2013167. https:\/\/doi.org\/10.1016\/j.ccr.2005.06.015","journal-title":"Cancer Cell"},{"issue":"2","key":"10009_CR39","doi-asserted-by":"publisher","first-page":"213","DOI":"10.1016\/j.molcel.2019.10.023","volume":"77","author":"JY Wu","year":"2020","unstructured":"Wu, J. Y., Huang, T. W., Hsieh, Y. T., Wang, Y. F., Yen, C. C., Lee, G. L., et al. (2020). Cancer-derived succinate promotes macrophage polarization and cancer metastasis via succinate receptor. Molecular Cell, 77(2), 213-227 e215. https:\/\/doi.org\/10.1016\/j.molcel.2019.10.023","journal-title":"Molecular Cell"},{"issue":"8","key":"10009_CR40","doi-asserted-by":"publisher","first-page":"1086","DOI":"10.1016\/j.bbabio.2016.03.012","volume":"1857","author":"L Tretter","year":"2016","unstructured":"Tretter, L., Patocs, A., & Chinopoulos, C. (2016). Succinate, an intermediate in metabolism, signal transduction, ROS, hypoxia, and tumorigenesis. Biochimica et Biophysica Acta, 1857(8), 1086\u20131101. https:\/\/doi.org\/10.1016\/j.bbabio.2016.03.012","journal-title":"Biochimica et Biophysica Acta"},{"issue":"6988","key":"10009_CR41","doi-asserted-by":"publisher","first-page":"188","DOI":"10.1038\/nature02488","volume":"429","author":"W He","year":"2004","unstructured":"He, W., Miao, F. J., Lin, D. C., Schwandner, R. T., Wang, Z., Gao, J., et al. (2004). Citric acid cycle intermediates as ligands for orphan G-protein-coupled receptors. Nature, 429(6988), 188\u2013193. https:\/\/doi.org\/10.1038\/nature02488","journal-title":"Nature"},{"issue":"11","key":"10009_CR42","doi-asserted-by":"publisher","first-page":"4058","DOI":"10.1158\/0008-5472.CAN-07-5580","volume":"68","author":"AM Cervera","year":"2008","unstructured":"Cervera, A. M., Apostolova, N., Crespo, F. L., Mata, M., & McCreath, K. J. (2008). Cells silenced for SDHB expression display characteristic features of the tumor phenotype. Cancer Research, 68(11), 4058\u20134067. https:\/\/doi.org\/10.1158\/0008-5472.CAN-07-5580","journal-title":"Cancer Research"},{"issue":"10","key":"10009_CR43","doi-asserted-by":"publisher","first-page":"1067","DOI":"10.1038\/nm.1873","volume":"14","author":"P Sapieha","year":"2008","unstructured":"Sapieha, P., Sirinyan, M., Hamel, D., Zaniolo, K., Joyal, J. S., Cho, J. H., et al. (2008). The succinate receptor GPR91 in neurons has a major role in retinal angiogenesis. Nature Medicine, 14(10), 1067\u20131076. https:\/\/doi.org\/10.1038\/nm.1873","journal-title":"Nature Medicine"},{"issue":"8","key":"10009_CR44","doi-asserted-by":"publisher","first-page":"13174","DOI":"10.18632\/oncotarget.14485","volume":"8","author":"X Mu","year":"2017","unstructured":"Mu, X., Zhao, T., Xu, C., Shi, W., Geng, B., Shen, J., et al. (2017). Oncometabolite succinate promotes angiogenesis by upregulating VEGF expression through GPR91-mediated STAT3 and ERK activation. Oncotarget, 8(8), 13174\u201313185. https:\/\/doi.org\/10.18632\/oncotarget.14485","journal-title":"Oncotarget"},{"issue":"32","key":"10009_CR45","doi-asserted-by":"publisher","first-page":"53819","DOI":"10.18632\/oncotarget.17734","volume":"8","author":"T Zhao","year":"2017","unstructured":"Zhao, T., Mu, X., & You, Q. (2017). Succinate: An initiator in tumorigenesis and progression. Oncotarget, 8(32), 53819\u201353828. https:\/\/doi.org\/10.18632\/oncotarget.17734","journal-title":"Oncotarget"},{"issue":"7444","key":"10009_CR46","doi-asserted-by":"publisher","first-page":"238","DOI":"10.1038\/nature11986","volume":"496","author":"GM Tannahill","year":"2013","unstructured":"Tannahill, G. M., Curtis, A. M., Adamik, J., Palsson-McDermott, E. M., McGettrick, A. F., Goel, G., et al. (2013). Succinate is an inflammatory signal that induces IL-1beta through HIF-1alpha. Nature, 496(7444), 238\u2013242. https:\/\/doi.org\/10.1038\/nature11986","journal-title":"Nature"},{"issue":"5","key":"10009_CR47","doi-asserted-by":"publisher","first-page":"646","DOI":"10.1016\/j.cell.2011.02.013","volume":"144","author":"D Hanahan","year":"2011","unstructured":"Hanahan, D., & Weinberg, R. A. (2011). Hallmarks of cancer: The next generation. Cell, 144(5), 646\u2013674. https:\/\/doi.org\/10.1016\/j.cell.2011.02.013","journal-title":"Cell"},{"issue":"1","key":"10009_CR48","doi-asserted-by":"publisher","first-page":"15","DOI":"10.1084\/jem.20151570","volume":"213","author":"LA O'Neill","year":"2016","unstructured":"O\u2019Neill, L. A., & Pearce, E. J. (2016). Immunometabolism governs dendritic cell and macrophage function. Journal of Experimental Medicine, 213(1), 15\u201323. https:\/\/doi.org\/10.1084\/jem.20151570","journal-title":"Journal of Experimental Medicine"},{"issue":"1","key":"10009_CR49","doi-asserted-by":"publisher","first-page":"36","DOI":"10.3803\/EnM.2020.35.1.36","volume":"35","author":"Y Guo","year":"2020","unstructured":"Guo, Y., Cho, S. W., Saxena, D., & Li, X. (2020). Multifaceted actions of succinate as a signaling transmitter vary with its cellular locations. Endocrinol Metab (Seoul), 35(1), 36\u201343. https:\/\/doi.org\/10.3803\/EnM.2020.35.1.36","journal-title":"Endocrinol Metab (Seoul)"},{"issue":"6487","key":"10009_CR50","doi-asserted-by":"publisher","first-page":"eaaw5473","DOI":"10.1126\/science.aaw5473","volume":"368","author":"B Faubert","year":"2020","unstructured":"Faubert, B., Solmonson, A., & DeBerardinis, R. J. (2020). Metabolic reprogramming and cancer progression. Science, 368(6487), eaaw5473. https:\/\/doi.org\/10.1126\/science.aaw5473","journal-title":"Science"},{"issue":"3","key":"10009_CR51","doi-asserted-by":"publisher","first-page":"883","DOI":"10.14670\/HH-19.883","volume":"19","author":"AS Tischler","year":"2004","unstructured":"Tischler, A. S., Powers, J. F., & Alroy, J. (2004). Animal models of pheochromocytoma. Histology and Histopathology, 19(3), 883\u2013895. https:\/\/doi.org\/10.14670\/HH-19.883","journal-title":"Histology and Histopathology"},{"issue":"12","key":"10009_CR52","doi-asserted-by":"publisher","first-page":"R433","DOI":"10.1530\/ERC-19-0434","volume":"27","author":"JP Bayley","year":"2020","unstructured":"Bayley, J. P., & Devilee, P. (2020). Advances in paraganglioma-pheochromocytoma cell lines and xenografts. Endocrine-Related Cancer, 27(12), R433\u2013R450. https:\/\/doi.org\/10.1530\/ERC-19-0434","journal-title":"Endocrine-Related Cancer"},{"issue":"4","key":"10009_CR53","doi-asserted-by":"publisher","first-page":"575","DOI":"10.1007\/s00401-013-1165-y","volume":"126","author":"A Cama","year":"2013","unstructured":"Cama, A., Verginelli, F., Lotti, L. V., Napolitano, F., Morgano, A., D\u2019Orazio, A., et al. (2013). Integrative genetic, epigenetic and pathological analysis of paraganglioma reveals complex dysregulation of NOTCH signaling. Acta Neuropathologica, 126(4), 575\u2013594. https:\/\/doi.org\/10.1007\/s00401-013-1165-y","journal-title":"Acta Neuropathologica"},{"issue":"6","key":"10009_CR54","doi-asserted-by":"publisher","first-page":"e0178995","DOI":"10.1371\/journal.pone.0178995","volume":"12","author":"R Florio","year":"2017","unstructured":"Florio, R., De Lellis, L., di Giacomo, V., Di Marcantonio, M. C., Cristiano, L., Basile, M., et al. (2017). Effects of PPARalpha inhibition in head and neck paraganglioma cells. PLoS ONE, 12(6), e0178995. https:\/\/doi.org\/10.1371\/journal.pone.0178995","journal-title":"PLoS ONE"},{"issue":"32","key":"10009_CR55","doi-asserted-by":"publisher","first-page":"32955","DOI":"10.18632\/oncotarget.5106","volume":"6","author":"C Loriot","year":"2015","unstructured":"Loriot, C., Domingues, M., Berger, A., Menara, M., Ruel, M., Morin, A., et al. (2015). Deciphering the molecular basis of invasiveness in Sdhb-deficient cells. Oncotarget, 6(32), 32955\u201332965. https:\/\/doi.org\/10.18632\/oncotarget.5106","journal-title":"Oncotarget"},{"issue":"1","key":"10009_CR56","doi-asserted-by":"publisher","first-page":"303","DOI":"10.1096\/fj.201901456R","volume":"34","author":"K Kluckova","year":"2020","unstructured":"Kluckova, K., Thakker, A., Vettore, L., Escribano-Gonzalez, C., Hindshaw, R. L., Tearle, J. L. E., et al. (2020). Succinate dehydrogenase deficiency in a chromaffin cell model retains metabolic fitness through the maintenance of mitochondrial NADH oxidoreductase function. The FASEB Journal, 34(1), 303\u2013315. https:\/\/doi.org\/10.1096\/fj.201901456R","journal-title":"The FASEB Journal"},{"issue":"6","key":"10009_CR57","doi-asserted-by":"publisher","first-page":"337","DOI":"10.1530\/ERC-19-0474","volume":"27","author":"JF Powers","year":"2020","unstructured":"Powers, J. F., Cochran, B., Baleja, J. D., Sikes, H. D., Pattison, A. D., Zhang, X., et al. (2020). A xenograft and cell line model of SDH-deficient pheochromocytoma derived from Sdhb+\/- rats. Endocrine-Related Cancer, 27(6), 337\u2013354. https:\/\/doi.org\/10.1530\/ERC-19-0474","journal-title":"Endocrine-Related Cancer"},{"issue":"7","key":"10009_CR58","doi-asserted-by":"publisher","first-page":"3470","DOI":"10.1128\/MCB.15.7.3470","volume":"15","author":"R Hopewell","year":"1995","unstructured":"Hopewell, R., & Ziff, E. B. (1995). The nerve growth factor-responsive PC12 cell line does not express the Myc dimerization partner Max. Molecular and Cellular Biology, 15(7), 3470\u20133478. https:\/\/doi.org\/10.1128\/MCB.15.7.3470","journal-title":"Molecular and Cellular Biology"},{"issue":"7","key":"10009_CR59","doi-asserted-by":"publisher","first-page":"2424","DOI":"10.1073\/pnas.73.7.2424","volume":"73","author":"LA Greene","year":"1976","unstructured":"Greene, L. A., & Tischler, A. S. (1976). Establishment of a noradrenergic clonal line of rat adrenal pheochromocytoma cells which respond to nerve growth factor. Proceedings of the National Academy of Sciences, 73(7), 2424\u20132428. https:\/\/doi.org\/10.1073\/pnas.73.7.2424","journal-title":"Proceedings of the National Academy of Sciences"},{"issue":"14","key":"10009_CR60","doi-asserted-by":"publisher","first-page":"3868","DOI":"10.1158\/1078-0432.CCR-19-2335","volume":"26","author":"Y Liu","year":"2020","unstructured":"Liu, Y., Pang, Y., Zhu, B., Uher, O., Caisova, V., Huynh, T. T., et al. (2020). Therapeutic targeting of SDHB-mutated pheochromocytoma\/paraganglioma with pharmacologic ascorbic acid. Clinical Cancer Research, 26(14), 3868\u20133880. https:\/\/doi.org\/10.1158\/1078-0432.CCR-19-2335","journal-title":"Clinical Cancer Research"},{"issue":"3","key":"10009_CR61","doi-asserted-by":"publisher","first-page":"309","DOI":"10.1007\/s004410000290","volume":"302","author":"JF Powers","year":"2000","unstructured":"Powers, J. F., Evinger, M. J., Tsokas, P., Bedri, S., Alroy, J., Shahsavari, M., et al. (2000). Pheochromocytoma cell lines from heterozygous neurofibromatosis knockout mice. Cell and Tissue Research, 302(3), 309\u2013320. https:\/\/doi.org\/10.1007\/s004410000290","journal-title":"Cell and Tissue Research"},{"issue":"14","key":"10009_CR62","doi-asserted-by":"publisher","first-page":"3423","DOI":"10.1158\/1078-0432.CCR-17-3406","volume":"24","author":"Y Pang","year":"2018","unstructured":"Pang, Y., Lu, Y., Caisova, V., Liu, Y., Bullova, P., Huynh, T. T., et al. (2018). Targeting NAD(+)\/PARP DNA repair pathway as a novel therapeutic approach to SDHB-mutated cluster I pheochromocytoma and paraganglioma. Clinical Cancer Research, 24(14), 3423\u20133432. https:\/\/doi.org\/10.1158\/1078-0432.CCR-17-3406","journal-title":"Clinical Cancer Research"},{"issue":"3","key":"10009_CR63","doi-asserted-by":"publisher","first-page":"239","DOI":"10.1007\/s10585-009-9236-0","volume":"26","author":"L Martiniova","year":"2009","unstructured":"Martiniova, L., Lai, E. W., Elkahloun, A. G., Abu-Asab, M., Wickremasinghe, A., Solis, D. C., et al. (2009). Characterization of an animal model of aggressive metastatic pheochromocytoma linked to a specific gene signature. Clinical & Experimental Metastasis, 26(3), 239\u2013250. https:\/\/doi.org\/10.1007\/s10585-009-9236-0","journal-title":"Clinical & Experimental Metastasis"},{"issue":"6","key":"10009_CR64","doi-asserted-by":"publisher","first-page":"e65624","DOI":"10.1371\/journal.pone.0065624","volume":"8","author":"HK Ghayee","year":"2013","unstructured":"Ghayee, H. K., Bhagwandin, V. J., Stastny, V., Click, A., Ding, L. H., Mizrachi, D., et al. (2013). Progenitor cell line (hPheo1) derived from a human pheochromocytoma tumor. PLoS ONE, 8(6), e65624. https:\/\/doi.org\/10.1371\/journal.pone.0065624","journal-title":"PLoS ONE"},{"issue":"26","key":"10009_CR65","doi-asserted-by":"publisher","first-page":"40531","DOI":"10.18632\/oncotarget.9643","volume":"7","author":"P Bullova","year":"2016","unstructured":"Bullova, P., Cougnoux, A., Abunimer, L., Kopacek, J., Pastorekova, S., & Pacak, K. (2016). Hypoxia potentiates the cytotoxic effect of piperlongumine in pheochromocytoma models. Oncotarget, 7(26), 40531\u201340545. https:\/\/doi.org\/10.18632\/oncotarget.9643","journal-title":"Oncotarget"},{"issue":"4","key":"10009_CR66","doi-asserted-by":"publisher","first-page":"696","DOI":"10.1007\/s11064-015-1738-3","volume":"41","author":"Y Saito","year":"2016","unstructured":"Saito, Y., Ishii, K. A., Aita, Y., Ikeda, T., Kawakami, Y., Shimano, H., et al. (2016). Loss of SDHB elevates catecholamine synthesis and secretion depending on ROS production and HIF stabilization. Neurochemical Research, 41(4), 696\u2013706. https:\/\/doi.org\/10.1007\/s11064-015-1738-3","journal-title":"Neurochemical Research"},{"issue":"3","key":"10009_CR67","doi-asserted-by":"publisher","first-page":"473","DOI":"10.1007\/s00441-018-2907-x","volume":"374","author":"S Richter","year":"2018","unstructured":"Richter, S., D\u2019Antongiovanni, V., Martinelli, S., Bechmann, N., Riverso, M., Poitz, D. M., et al. (2018). Primary fibroblast co-culture stimulates growth and metabolism in Sdhb-impaired mouse pheochromocytoma MTT cells. Cell and Tissue Research, 374(3), 473\u2013485. https:\/\/doi.org\/10.1007\/s00441-018-2907-x","journal-title":"Cell and Tissue Research"},{"key":"10009_CR68","doi-asserted-by":"publisher","first-page":"154297","DOI":"10.1016\/j.metabol.2020.154297","volume":"110","author":"SK Rai","year":"2020","unstructured":"Rai, S. K., Bril, F., Hatch, H. M., Xu, Y., Shelton, L., Kalavalapalli, S., et al. (2020). Targeting pheochromocytoma\/paraganglioma with polyamine inhibitors. Metabolism, 110, 154297. https:\/\/doi.org\/10.1016\/j.metabol.2020.154297","journal-title":"Metabolism"},{"issue":"1","key":"10009_CR69","doi-asserted-by":"crossref","first-page":"203","DOI":"10.1158\/0008-5472.203.65.1","volume":"65","author":"T Ishii","year":"2005","unstructured":"Ishii, T., Yasuda, K., Akatsuka, A., Hino, O., Hartman, P. S., & Ishii, N. (2005). A mutation in the SDHC gene of complex II increases oxidative stress, resulting in apoptosis and tumorigenesis. Cancer Research, 65(1), 203\u2013209.","journal-title":"Cancer Research"},{"key":"10009_CR70","doi-asserted-by":"publisher","first-page":"89","DOI":"10.1186\/1476-4598-8-89","volume":"8","author":"AM Cervera","year":"2009","unstructured":"Cervera, A. M., Bayley, J. P., Devilee, P., & McCreath, K. J. (2009). Inhibition of succinate dehydrogenase dysregulates histone modification in mammalian cells. Molecular Cancer, 8, 89. https:\/\/doi.org\/10.1186\/1476-4598-8-89","journal-title":"Molecular Cancer"},{"issue":"5","key":"10009_CR71","doi-asserted-by":"publisher","first-page":"e0127471","DOI":"10.1371\/journal.pone.0127471","volume":"10","author":"YF Her","year":"2015","unstructured":"Her, Y. F., Nelson-Holte, M., & Maher, L. J., 3rd. (2015). Oxygen concentration controls epigenetic effects in models of familial paraganglioma. PLoS ONE, 10(5), e0127471. https:\/\/doi.org\/10.1371\/journal.pone.0127471","journal-title":"PLoS ONE"},{"issue":"17","key":"10009_CR72","doi-asserted-by":"publisher","first-page":"28922","DOI":"10.18632\/oncotarget.15959","volume":"8","author":"S Kitazawa","year":"2017","unstructured":"Kitazawa, S., Ebara, S., Ando, A., Baba, Y., Satomi, Y., Soga, T., et al. (2017). Succinate dehydrogenase B-deficient cancer cells are highly sensitive to bromodomain and extra-terminal inhibitors. Oncotarget, 8(17), 28922\u201328938. https:\/\/doi.org\/10.18632\/oncotarget.15959","journal-title":"Oncotarget"},{"issue":"3","key":"10009_CR73","doi-asserted-by":"publisher","first-page":"353","DOI":"10.1038\/ng0794-353","volume":"7","author":"T Jacks","year":"1994","unstructured":"Jacks, T., Shih, T. S., Schmitt, E. M., Bronson, R. T., Bernards, A., & Weinberg, R. A. (1994). Tumour predisposition in mice heterozygous for a targeted mutation in Nf1. Nature Genetics, 7(3), 353\u2013361. https:\/\/doi.org\/10.1038\/ng0794-353","journal-title":"Nature Genetics"},{"issue":"1","key":"10009_CR74","doi-asserted-by":"publisher","first-page":"e85528","DOI":"10.1371\/journal.pone.0085528","volume":"9","author":"A Millan-Ucles","year":"2014","unstructured":"Millan-Ucles, A., Diaz-Castro, B., Garcia-Flores, P., Baez, A., Perez-Simon, J. A., Lopez-Barneo, J., et al. (2014). A conditional mouse mutant in the tumor suppressor SdhD gene unveils a link between p21(WAF1\/Cip1) induction and mitochondrial dysfunction. PLoS ONE, 9(1), e85528. https:\/\/doi.org\/10.1371\/journal.pone.0085528","journal-title":"PLoS ONE"},{"issue":"4","key":"10009_CR75","doi-asserted-by":"publisher","first-page":"6700","DOI":"10.18632\/oncotarget.14265","volume":"8","author":"A Merlo","year":"2017","unstructured":"Merlo, A., Bernardo-Castineira, C., Saenz-de-Santa-Maria, I., Pitiot, A. S., Balbin, M., Astudillo, A., et al. (2017). Role of VHL, HIF1A and SDH on the expression of miR-210: Implications for tumoral pseudo-hypoxic fate. Oncotarget, 8(4), 6700\u20136717. https:\/\/doi.org\/10.18632\/oncotarget.14265","journal-title":"Oncotarget"},{"issue":"3","key":"10009_CR76","doi-asserted-by":"publisher","first-page":"599","DOI":"10.3390\/cancers12030599","volume":"12","author":"B Sarkadi","year":"2020","unstructured":"Sarkadi, B., Meszaros, K., Krencz, I., Canu, L., Krokker, L., Zakarias, S., et al. (2020). Glutaminases as a novel target for SDHB-associated pheochromocytomas\/paragangliomas. Cancers (Basel), 12(3), 599. https:\/\/doi.org\/10.3390\/cancers12030599","journal-title":"Cancers (Basel)"},{"issue":"1","key":"10009_CR77","doi-asserted-by":"publisher","first-page":"151057","DOI":"10.1016\/j.ejcb.2019.151057","volume":"99","author":"B Moosavi","year":"2020","unstructured":"Moosavi, B., Zhu, X. L., Yang, W. C., & Yang, G. F. (2020). Molecular pathogenesis of tumorigenesis caused by succinate dehydrogenase defect. European Journal of Cell Biology, 99(1), 151057. https:\/\/doi.org\/10.1016\/j.ejcb.2019.151057","journal-title":"European Journal of Cell Biology"},{"issue":"10","key":"10009_CR78","doi-asserted-by":"publisher","first-page":"1860","DOI":"10.1093\/hmg\/ddp102","volume":"18","author":"P Goffrini","year":"2009","unstructured":"Goffrini, P., Ercolino, T., Panizza, E., Giache, V., Cavone, L., Chiarugi, A., et al. (2009). Functional study in a yeast model of a novel succinate dehydrogenase subunit B gene germline missense mutation (C191Y) diagnosed in a patient affected by a glomus tumor. Human Molecular Genetics, 18(10), 1860\u20131868. https:\/\/doi.org\/10.1093\/hmg\/ddp102","journal-title":"Human Molecular Genetics"},{"issue":"2","key":"10009_CR79","doi-asserted-by":"publisher","first-page":"e56827","DOI":"10.1371\/journal.pone.0056827","volume":"8","author":"I Bancos","year":"2013","unstructured":"Bancos, I., Bida, J. P., Tian, D., Bundrick, M., John, K., Holte, M. N., et al. (2013). High-throughput screening for growth inhibitors using a yeast model of familial paraganglioma. PLoS ONE, 8(2), e56827. https:\/\/doi.org\/10.1371\/journal.pone.0056827","journal-title":"PLoS ONE"},{"issue":"4","key":"10009_CR80","doi-asserted-by":"publisher","first-page":"804","DOI":"10.1093\/hmg\/dds487","volume":"22","author":"E Panizza","year":"2013","unstructured":"Panizza, E., Ercolino, T., Mori, L., Rapizzi, E., Castellano, M., Opocher, G., et al. (2013). Yeast model for evaluating the pathogenic significance of SDHB, SDHC and SDHD mutations in PHEO-PGL syndrome. Human Molecular Genetics, 22(4), 804\u2013815. https:\/\/doi.org\/10.1093\/hmg\/dds487","journal-title":"Human Molecular Genetics"},{"issue":"6","key":"10009_CR81","doi-asserted-by":"publisher","first-page":"654","DOI":"10.1038\/ng.378","volume":"41","author":"D Ghezzi","year":"2009","unstructured":"Ghezzi, D., Goffrini, P., Uziel, G., Horvath, R., Klopstock, T., Lochmuller, H., et al. (2009). SDHAF1, encoding a LYR complex-II specific assembly factor, is mutated in SDH-defective infantile leukoencephalopathy. Nature Genetics, 41(6), 654\u2013656. https:\/\/doi.org\/10.1038\/ng.378","journal-title":"Nature Genetics"},{"issue":"2","key":"10009_CR82","doi-asserted-by":"publisher","first-page":"241","DOI":"10.1016\/j.cmet.2014.05.012","volume":"20","author":"JG Van Vranken","year":"2014","unstructured":"Van Vranken, J. G., Bricker, D. K., Dephoure, N., Gygi, S. P., Cox, J. E., Thummel, C. S., et al. (2014). SDHAF4 promotes mitochondrial succinate dehydrogenase activity and prevents neurodegeneration. Cell Metabolism, 20(2), 241\u2013252. https:\/\/doi.org\/10.1016\/j.cmet.2014.05.012","journal-title":"Cell Metabolism"},{"issue":"3","key":"10009_CR83","doi-asserted-by":"publisher","first-page":"559","DOI":"10.1016\/j.jmb.2009.02.028","volume":"387","author":"J Huang","year":"2009","unstructured":"Huang, J., & Lemire, B. D. (2009). Mutations in the C. elegans succinate dehydrogenase iron-sulfur subunit promote superoxide generation and premature aging. J Mol Biol, 387(3), 559\u2013569. https:\/\/doi.org\/10.1016\/j.jmb.2009.02.028","journal-title":"J Mol Biol"},{"key":"10009_CR84","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1242\/dmm.044925","volume":"13","author":"E Saskoi","year":"2020","unstructured":"Saskoi, E., Hujber, Z., Nyiro, G., Liko, I., Matyasi, B., Petovari, G., et al. (2020). The SDHB Arg230His mutation causing familial paraganglioma alters glycolysis in a new Caenorhabditis elegans model. Disease Models and Mechanisms, 13, 1\u201316.","journal-title":"Disease Models and Mechanisms"},{"issue":"12","key":"10009_CR85","doi-asserted-by":"publisher","first-page":"e0227033","DOI":"10.1371\/journal.pone.0227033","volume":"14","author":"MM Braun","year":"2019","unstructured":"Braun, M. M., Damjanac, T., Zhang, Y., Chen, C., Hu, J., & Maher, L. J., 3rd. (2019). Modeling succinate dehydrogenase loss disorders in C. elegans through effects on hypoxia-inducible factor. PLoS One, 14(12), e0227033. https:\/\/doi.org\/10.1371\/journal.pone.0227033","journal-title":"PLoS One"},{"issue":"3\u20134","key":"10009_CR86","doi-asserted-by":"publisher","first-page":"165","DOI":"10.1016\/0921-8734(90)90022-j","volume":"237","author":"N Ishii","year":"1990","unstructured":"Ishii, N., Takahashi, K., Tomita, S., Keino, T., Honda, S., Yoshino, K., et al. (1990). A methyl viologen-sensitive mutant of the nematode Caenorhabditis elegans. Mutation Research, 237(3\u20134), 165\u2013171. https:\/\/doi.org\/10.1016\/0921-8734(90)90022-j","journal-title":"Mutation Research"},{"issue":"6694","key":"10009_CR87","doi-asserted-by":"publisher","first-page":"694","DOI":"10.1038\/29331","volume":"394","author":"N Ishii","year":"1998","unstructured":"Ishii, N., Fujii, M., Hartman, P. S., Tsuda, M., Yasuda, K., Senoo-Matsuda, N., et al. (1998). A mutation in succinate dehydrogenase cytochrome b causes oxidative stress and ageing in nematodes. Nature, 394(6694), 694\u2013697. https:\/\/doi.org\/10.1038\/29331","journal-title":"Nature"},{"issue":"5","key":"10009_CR88","doi-asserted-by":"publisher","first-page":"588","DOI":"10.1016\/j.bbabio.2012.10.016","volume":"1827","author":"T Ishii","year":"2013","unstructured":"Ishii, T., Miyazawa, M., Onouchi, H., Yasuda, K., Hartman, P. S., & Ishii, N. (2013). Model animals for the study of oxidative stress from complex II. Biochimica et Biophysica Acta, 1827(5), 588\u2013597. https:\/\/doi.org\/10.1016\/j.bbabio.2012.10.016","journal-title":"Biochimica et Biophysica Acta"},{"issue":"4","key":"10009_CR89","doi-asserted-by":"publisher","first-page":"B240","DOI":"10.1093\/gerona\/53a.4.b240","volume":"53","author":"H Adachi","year":"1998","unstructured":"Adachi, H., Fujiwara, Y., & Ishii, N. (1998). Effects of oxygen on protein carbonyl and aging in Caenorhabditis elegans mutants with long (age-1) and short (mev-1) life spans. Journals of Gerontology. Series A, Biological Sciences and Medical Sciences, 53(4), B240-244. https:\/\/doi.org\/10.1093\/gerona\/53a.4.b240","journal-title":"Journals of Gerontology. Series A, Biological Sciences and Medical Sciences"},{"issue":"4","key":"10009_CR90","doi-asserted-by":"publisher","first-page":"100990","DOI":"10.1016\/j.isci.2020.100990","volume":"23","author":"J Goncalves","year":"2020","unstructured":"Goncalves, J., Wan, Y., Guo, X., Rha, K., LeBoeuf, B., Zhang, L., et al. (2020). Succinate dehydrogenase-regulated phosphoenolpyruvate carboxykinase sustains copulation fitness in aging C. elegans males. iScience, 23(4), 100990. https:\/\/doi.org\/10.1016\/j.isci.2020.100990","journal-title":"iScience"},{"issue":"44","key":"10009_CR91","doi-asserted-by":"publisher","first-page":"16382","DOI":"10.1073\/pnas.0607918103","volume":"103","author":"DW Walker","year":"2006","unstructured":"Walker, D. W., Hajek, P., Muffat, J., Knoepfle, D., Cornelison, S., Attardi, G., et al. (2006). Hypersensitivity to oxygen and shortened lifespan in a Drosophila mitochondrial complex II mutant. Proceedings of the National Academy of Sciences, 103(44), 16382\u201316387. https:\/\/doi.org\/10.1073\/pnas.0607918103","journal-title":"Proceedings of the National Academy of Sciences"},{"issue":"2","key":"10009_CR92","doi-asserted-by":"publisher","first-page":"e01217","DOI":"10.1016\/j.heliyon.2019.e01217","volume":"5","author":"F Fan","year":"2019","unstructured":"Fan, F., Sam, R., Ryan, E., Alvarado, K., & Villa-Cuesta, E. (2019). Rapamycin as a potential treatment for succinate dehydrogenase deficiency. Heliyon, 5(2), e01217. https:\/\/doi.org\/10.1016\/j.heliyon.2019.e01217","journal-title":"Heliyon"},{"issue":"2","key":"10009_CR93","doi-asserted-by":"publisher","first-page":"253","DOI":"10.1016\/j.cmet.2014.05.014","volume":"20","author":"U Na","year":"2014","unstructured":"Na, U., Yu, W., Cox, J., Bricker, D. K., Brockmann, K., Rutter, J., et al. (2014). The LYR factors SDHAF1 and SDHAF3 mediate maturation of the iron-sulfur subunit of succinate dehydrogenase. Cell Metabolism, 20(2), 253\u2013266. https:\/\/doi.org\/10.1016\/j.cmet.2014.05.014","journal-title":"Cell Metabolism"},{"issue":"1","key":"10009_CR94","doi-asserted-by":"publisher","first-page":"65","DOI":"10.1530\/ERC-20-0308","volume":"28","author":"M Dona","year":"2021","unstructured":"Dona, M., Waaijers, S., Richter, S., Eisenhofer, G., Korving, J., Kamel, S. M., et al. (2021). Loss of sdhb in zebrafish larvae recapitulates human paraganglioma characteristics. Endocrine-Related Cancer, 28(1), 65\u201377. https:\/\/doi.org\/10.1530\/ERC-20-0308","journal-title":"Endocrine-Related Cancer"},{"key":"10009_CR95","doi-asserted-by":"publisher","first-page":"69","DOI":"10.1186\/1750-1172-7-69","volume":"7","author":"A Ohlenbusch","year":"2012","unstructured":"Ohlenbusch, A., Edvardson, S., Skorpen, J., Bjornstad, A., Saada, A., Elpeleg, O., et al. (2012). Leukoencephalopathy with accumulated succinate is indicative of SDHAF1 related complex II deficiency. Orphanet Journal of Rare Diseases, 7, 69. https:\/\/doi.org\/10.1186\/1750-1172-7-69","journal-title":"Orphanet Journal of Rare Diseases"},{"key":"10009_CR96","doi-asserted-by":"publisher","first-page":"e19887","DOI":"10.7554\/eLife.19887","volume":"5","author":"MA Lim","year":"2016","unstructured":"Lim, M. A., Chitturi, J., Laskova, V., Meng, J., Findeis, D., Wiekenberg, A., et al. (2016). Neuroendocrine modulation sustains the C. elegans forward motor state. Elife, 5, e19887. https:\/\/doi.org\/10.7554\/eLife.19887","journal-title":"Elife"},{"issue":"1","key":"10009_CR97","doi-asserted-by":"publisher","first-page":"155","DOI":"10.1016\/j.mito.2010.09.006","volume":"11","author":"T Ishii","year":"2011","unstructured":"Ishii, T., Miyazawa, M., Onodera, A., Yasuda, K., Kawabe, N., Kirinashizawa, M., et al. (2011). Mitochondrial reactive oxygen species generation by the SDHC V69E mutation causes low birth weight and neonatal growth retardation. Mitochondrion, 11(1), 155\u2013165. https:\/\/doi.org\/10.1016\/j.mito.2010.09.006","journal-title":"Mitochondrion"},{"issue":"6","key":"10009_CR98","doi-asserted-by":"publisher","first-page":"858","DOI":"10.1002\/ps.3241","volume":"68","author":"T Veloukas","year":"2012","unstructured":"Veloukas, T., & Karaoglanidis, G. S. (2012). Biological activity of the succinate dehydrogenase inhibitor fluopyram against Botrytis cinerea and fungal baseline sensitivity. Pest Management Science, 68(6), 858\u2013864. https:\/\/doi.org\/10.1002\/ps.3241","journal-title":"Pest Management Science"},{"issue":"6","key":"10009_CR99","doi-asserted-by":"publisher","first-page":"1347","DOI":"10.1007\/s00294-019-01001-w","volume":"65","author":"L Duvenage","year":"2019","unstructured":"Duvenage, L., Munro, C. A., & Gourlay, C. W. (2019). The potential of respiration inhibition as a new approach to combat human fungal pathogens. Current Genetics, 65(6), 1347\u20131353. https:\/\/doi.org\/10.1007\/s00294-019-01001-w","journal-title":"Current Genetics"},{"key":"10009_CR100","doi-asserted-by":"publisher","first-page":"29695","DOI":"10.1038\/srep29695","volume":"6","author":"AP Wojtovich","year":"2016","unstructured":"Wojtovich, A. P., Wei, A. Y., Sherman, T. A., Foster, T. H., & Nehrke, K. (2016). Chromophore-assisted light inactivation of mitochondrial electron transport chain complex II in Caenorhabditis elegans. Science and Reports, 6, 29695. https:\/\/doi.org\/10.1038\/srep29695","journal-title":"Science and Reports"},{"key":"10009_CR101","doi-asserted-by":"publisher","first-page":"656851","DOI":"10.3389\/fonc.2021.656851","volume":"11","author":"A Hipolito","year":"2021","unstructured":"Hipolito, A., Martins, F., Mendes, C., Lopes-Coelho, F., & Serpa, J. (2021). Molecular and metabolic reprogramming: Pulling the strings toward tumor metastasiS. Frontiers in Oncology, 11, 656851. https:\/\/doi.org\/10.3389\/fonc.2021.656851","journal-title":"Frontiers in Oncology"},{"issue":"16","key":"10009_CR102","doi-asserted-by":"publisher","first-page":"3347","DOI":"10.1128\/MCB.00128-12","volume":"32","author":"B Diaz-Castro","year":"2012","unstructured":"Diaz-Castro, B., Pintado, C. O., Garcia-Flores, P., Lopez-Barneo, J., & Piruat, J. I. (2012). Differential impairment of catecholaminergic cell maturation and survival by genetic mitochondrial complex II dysfunction. Molecular and Cellular Biology, 32(16), 3347\u20133357. https:\/\/doi.org\/10.1128\/MCB.00128-12","journal-title":"Molecular and Cellular Biology"},{"key":"10009_CR103","doi-asserted-by":"publisher","first-page":"40","DOI":"10.1016\/j.mce.2015.06.027","volume":"421","author":"C Lepoutre-Lussey","year":"2016","unstructured":"Lepoutre-Lussey, C., Thibault, C., Buffet, A., Morin, A., Badoual, C., Benit, P., et al. (2016). From Nf1 to Sdhb knockout: Successes and failures in the quest for animal models of pheochromocytoma. Molecular and Cellular Endocrinology, 421, 40\u201348. https:\/\/doi.org\/10.1016\/j.mce.2015.06.027","journal-title":"Molecular and Cellular Endocrinology"},{"issue":"1","key":"10009_CR104","doi-asserted-by":"publisher","first-page":"2","DOI":"10.1007\/s12022-016-9452-5","volume":"28","author":"JF Powers","year":"2017","unstructured":"Powers, J. F., Pacak, K., & Tischler, A. S. (2017). Pathology of human pheochromocytoma and paraganglioma xenografts in NSG mice. Endocrine Pathology, 28(1), 2\u20136. https:\/\/doi.org\/10.1007\/s12022-016-9452-5","journal-title":"Endocrine Pathology"},{"issue":"5","key":"10009_CR105","doi-asserted-by":"publisher","first-page":"1120","DOI":"10.1158\/1078-0432.CCR-15-1576","volume":"22","author":"C Lussey-Lepoutre","year":"2016","unstructured":"Lussey-Lepoutre, C., Bellucci, A., Morin, A., Buffet, A., Amar, L., Janin, M., et al. (2016). In vivo detection of succinate by magnetic resonance spectroscopy as a hallmark of SDHx mutations in paraganglioma. Clinical Cancer Research, 22(5), 1120\u20131129. https:\/\/doi.org\/10.1158\/1078-0432.CCR-15-1576.","journal-title":"Clinical Cancer Research"},{"issue":"8","key":"10009_CR106","doi-asserted-by":"publisher","first-page":"3518","DOI":"10.7150\/thno.40687","volume":"10","author":"C Facchin","year":"2020","unstructured":"Facchin, C., Perez-Liva, M., Garofalakis, A., Viel, T., Certain, A., Balvay, D., et al. (2020). Concurrent imaging of vascularization and metabolism in a mouse model of paraganglioma under anti-angiogenic treatment. Theranostics, 10(8), 3518\u20133532. https:\/\/doi.org\/10.7150\/thno.40687","journal-title":"Theranostics"},{"issue":"1","key":"10009_CR107","doi-asserted-by":"publisher","first-page":"126","DOI":"10.3390\/cancers13010126","volume":"13","author":"H Mohr","year":"2021","unstructured":"Mohr, H., Ballke, S., Bechmann, N., Gulde, S., Malekzadeh-Najafabadi, J., Peitzsch, M., et al. (2021). Mutation of the cell cycle regulator p27kip1 drives pseudohypoxic pheochromocytoma development. Cancers (Basel), 13(1), 126. https:\/\/doi.org\/10.3390\/cancers13010126","journal-title":"Cancers (Basel)"},{"issue":"Suppl 1","key":"10009_CR108","doi-asserted-by":"publisher","first-page":"13","DOI":"10.6061\/clinics\/2012(sup01)04","volume":"67","author":"NS Pellegata","year":"2012","unstructured":"Pellegata, N. S. (2012). MENX and MEN4. Clinics (S\u00e3o Paulo, Brazil), 67(Suppl 1), 13\u201318. https:\/\/doi.org\/10.6061\/clinics\/2012(sup01)04","journal-title":"Clinics (S\u00e3o Paulo, Brazil)"},{"issue":"24","key":"10009_CR109","doi-asserted-by":"publisher","first-page":"10933","DOI":"10.1128\/MCB.24.24.10933-10940.2004","volume":"24","author":"JI Piruat","year":"2004","unstructured":"Piruat, J. I., Pintado, C. O., Ortega-Saenz, P., Roche, M., & Lopez-Barneo, J. (2004). The mitochondrial SDHD gene is required for early embryogenesis, and its partial deficiency results in persistent carotid body glomus cell activation with full responsiveness to hypoxia. Molecular and Cellular Biology, 24(24), 10933\u201310940. https:\/\/doi.org\/10.1128\/MCB.24.24.10933-10940.2004","journal-title":"Molecular and Cellular Biology"},{"issue":"1","key":"10009_CR110","doi-asserted-by":"publisher","first-page":"59","DOI":"10.1093\/ilar\/ilu017","volume":"55","author":"BW Davis","year":"2014","unstructured":"Davis, B. W., & Ostrander, E. A. (2014). Domestic dogs and cancer research: A breed-based genomics approach. ILAR Journal, 55(1), 59\u201368. https:\/\/doi.org\/10.1093\/ilar\/ilu017","journal-title":"ILAR Journal"},{"issue":"5","key":"10009_CR111","doi-asserted-by":"publisher","first-page":"272","DOI":"10.1111\/j.1939-1676.1997.tb00464.x","volume":"11","author":"PY Barthez","year":"1997","unstructured":"Barthez, P. Y., Marks, S. L., Woo, J., Feldman, E. C., & Matteucci, M. (1997). Pheochromocytoma in dogs: 61 cases (1984\u20131995). Journal of Veterinary Internal Medicine, 11(5), 272\u2013278. https:\/\/doi.org\/10.1111\/j.1939-1676.1997.tb00464.x","journal-title":"Journal of Veterinary Internal Medicine"},{"issue":"4","key":"10009_CR112","doi-asserted-by":"publisher","first-page":"1158","DOI":"10.1111\/vco.12291","volume":"15","author":"S Galac","year":"2017","unstructured":"Galac, S., & Korpershoek, E. (2017). Pheochromocytomas and paragangliomas in humans and dogs. Veterinary and Comparative Oncology, 15(4), 1158\u20131170. https:\/\/doi.org\/10.1111\/vco.12291","journal-title":"Veterinary and Comparative Oncology"},{"issue":"1","key":"10009_CR113","doi-asserted-by":"publisher","first-page":"25","DOI":"10.1016\/j.jcpa.2014.03.007","volume":"151","author":"DE Holt","year":"2014","unstructured":"Holt, D. E., Henthorn, P., Howell, V. M., Robinson, B. G., & Benn, D. E. (2014). Succinate dehydrogenase subunit D and succinate dehydrogenase subunit B mutation analysis in canine phaeochromocytoma and paraganglioma. Journal of Comparative Pathology, 151(1), 25\u201334. https:\/\/doi.org\/10.1016\/j.jcpa.2014.03.007","journal-title":"Journal of Comparative Pathology"},{"issue":"2","key":"10009_CR114","doi-asserted-by":"publisher","first-page":"597","DOI":"10.1111\/jvim.12569","volume":"29","author":"E Salesov","year":"2015","unstructured":"Salesov, E., Boretti, F. S., Sieber-Ruckstuhl, N. S., Rentsch, K. M., Riond, B., Hofmann-Lehmann, R., et al. (2015). Urinary and plasma catecholamines and metanephrines in dogs with pheochromocytoma, hypercortisolism, nonadrenal disease and in healthy dogs. Journal of Veterinary Internal Medicine, 29(2), 597\u2013602. https:\/\/doi.org\/10.1111\/jvim.12569","journal-title":"Journal of Veterinary Internal Medicine"},{"issue":"5","key":"10009_CR115","doi-asserted-by":"publisher","first-page":"607","DOI":"10.3390\/cancers11050607","volume":"11","author":"E Korpershoek","year":"2019","unstructured":"Korpershoek, E., Dieduksman, D., Grinwis, G. C. M., Day, M. J., Reusch, C. E., Hilbe, M., et al. (2019). Molecular alterations in dog pheochromocytomas and paragangliomas. Cancers (Basel), 11(5), 607. https:\/\/doi.org\/10.3390\/cancers11050607","journal-title":"Cancers (Basel)"},{"issue":"1","key":"10009_CR116","doi-asserted-by":"publisher","first-page":"35","DOI":"10.1186\/s12917-020-2244-7","volume":"16","author":"S Lee","year":"2020","unstructured":"Lee, S., Lee, A., Chai, S. H., Lee, S., Kweon, O. K., & Kim, W. H. (2020). Ectopic Cushing\u2019s syndrome associated with a pheochromocytoma in a dog: A case report. BMC Veterinary Research, 16(1), 35. https:\/\/doi.org\/10.1186\/s12917-020-2244-7","journal-title":"BMC Veterinary Research"},{"issue":"9","key":"10009_CR117","doi-asserted-by":"publisher","first-page":"1683","DOI":"10.3390\/ani10091683","volume":"10","author":"FM Abed","year":"2020","unstructured":"Abed, F. M., Brown, M. A., Al-Mahmood, O. A., & Dark, M. J. (2020). SDHB and SDHA immunohistochemistry in canine pheochromocytomas. Animals (Basel), 10(9), 1683. https:\/\/doi.org\/10.3390\/ani10091683","journal-title":"Animals (Basel)"},{"issue":"2","key":"10009_CR118","doi-asserted-by":"publisher","first-page":"391","DOI":"10.1677\/ERC-08-0284","volume":"16","author":"HJ Timmers","year":"2009","unstructured":"Timmers, H. J., Gimenez-Roqueplo, A. P., Mannelli, M., & Pacak, K. (2009). Clinical aspects of SDHx-related pheochromocytoma and paraganglioma. Endocrine-Related Cancer, 16(2), 391\u2013400. https:\/\/doi.org\/10.1677\/ERC-08-0284","journal-title":"Endocrine-Related Cancer"},{"issue":"4","key":"10009_CR119","doi-asserted-by":"publisher","first-page":"267","DOI":"10.7326\/0003-4819-109-4-267","volume":"109","author":"SD Averbuch","year":"1988","unstructured":"Averbuch, S. D., Steakley, C. S., Young, R. C., Gelmann, E. P., Goldstein, D. S., Stull, R., et al. (1988). Malignant pheochromocytoma: Effective treatment with a combination of cyclophosphamide, vincristine, and dacarbazine. Annals of Internal Medicine, 109(4), 267\u2013273. https:\/\/doi.org\/10.7326\/0003-4819-109-4-267","journal-title":"Annals of Internal Medicine"},{"issue":"8","key":"10009_CR120","doi-asserted-by":"publisher","first-page":"2020","DOI":"10.1002\/cncr.23812","volume":"113","author":"H Huang","year":"2008","unstructured":"Huang, H., Abraham, J., Hung, E., Averbuch, S., Merino, M., Steinberg, S. M., et al. (2008). Treatment of malignant pheochromocytoma\/paraganglioma with cyclophosphamide, vincristine, and dacarbazine: Recommendation from a 22-year follow-up of 18 patients. Cancer, 113(8), 2020\u20132028. https:\/\/doi.org\/10.1002\/cncr.23812","journal-title":"Cancer"},{"issue":"2","key":"10009_CR121","doi-asserted-by":"publisher","first-page":"103","DOI":"10.1007\/s12672-013-0133-2","volume":"4","author":"A Tanabe","year":"2013","unstructured":"Tanabe, A., Naruse, M., Nomura, K., Tsuiki, M., Tsumagari, A., & Ichihara, A. (2013). Combination chemotherapy with cyclophosphamide, vincristine, and dacarbazine in patients with malignant pheochromocytoma and paraganglioma. Horm Cancer, 4(2), 103\u2013110. https:\/\/doi.org\/10.1007\/s12672-013-0133-2","journal-title":"Horm Cancer"},{"key":"10009_CR122","doi-asserted-by":"publisher","first-page":"465","DOI":"10.1196\/annals.1353.050","volume":"1073","author":"PA Fitzgerald","year":"2006","unstructured":"Fitzgerald, P. A., Goldsby, R. E., Huberty, J. P., Price, D. C., Hawkins, R. A., Veatch, J. J., et al. (2006). Malignant pheochromocytomas and paragangliomas: A phase II study of therapy with high-dose 131I-metaiodobenzylguanidine (131I-MIBG). Annals of the New York Academy of Sciences, 1073, 465\u2013490. https:\/\/doi.org\/10.1196\/annals.1353.050","journal-title":"Annals of the New York Academy of Sciences"},{"issue":"1","key":"10009_CR123","doi-asserted-by":"publisher","first-page":"7625","DOI":"10.1038\/s41598-019-43880-6","volume":"9","author":"H Wakabayashi","year":"2019","unstructured":"Wakabayashi, H., Inaki, A., Yoshimura, K., Murayama, T., Imai, Y., Higuchi, T., et al. (2019). A phase I clinical trial for [(131)I]meta-iodobenzylguanidine therapy in patients with refractory pheochromocytoma and paraganglioma. Science and Reports, 9(1), 7625. https:\/\/doi.org\/10.1038\/s41598-019-43880-6","journal-title":"Science and Reports"},{"issue":"11","key":"10009_CR124","doi-asserted-by":"publisher","first-page":"648","DOI":"10.1007\/BF03348026","volume":"20","author":"KC Loh","year":"1997","unstructured":"Loh, K. C., Fitzgerald, P. A., Matthay, K. K., Yeo, P. P., & Price, D. C. (1997). The treatment of malignant pheochromocytoma with iodine-131 metaiodobenzylguanidine (131I-MIBG): A comprehensive review of 116 reported patients. Journal of Endocrinological Investigation, 20(11), 648\u2013658. https:\/\/doi.org\/10.1007\/BF03348026","journal-title":"Journal of Endocrinological Investigation"},{"issue":"9","key":"10009_CR125","doi-asserted-by":"publisher","first-page":"839","DOI":"10.1007\/s12149-013-0755-z","volume":"27","author":"H Wakabayashi","year":"2013","unstructured":"Wakabayashi, H., Taki, J., Inaki, A., Nakamura, A., Kayano, D., Fukuoka, M., et al. (2013). Prognostic values of initial responses to low-dose (131)I-MIBG therapy in patients with malignant pheochromocytoma and paraganglioma. Annals of Nuclear Medicine, 27(9), 839\u2013846. https:\/\/doi.org\/10.1007\/s12149-013-0755-z","journal-title":"Annals of Nuclear Medicine"},{"issue":"10","key":"10009_CR126","doi-asserted-by":"publisher","first-page":"1505","DOI":"10.3390\/cancers11101505","volume":"11","author":"S Nolting","year":"2019","unstructured":"Nolting, S., Ullrich, M., Pietzsch, J., Ziegler, C. G., Eisenhofer, G., Grossman, A., et al. (2019). Current management of pheochromocytoma\/paraganglioma: A guide for the practicing clinician in the era of precision medicine. Cancers (Basel), 11(10), 1505. https:\/\/doi.org\/10.3390\/cancers11101505","journal-title":"Cancers (Basel)"},{"issue":"2","key":"10009_CR127","doi-asserted-by":"publisher","first-page":"280","DOI":"10.3390\/cancers12020280","volume":"12","author":"Y Liu","year":"2020","unstructured":"Liu, Y., Pang, Y., Caisova, V., Ding, J., Yu, D., Zhou, Y., et al. (2020). Targeting NRF2-governed glutathione synthesis for SDHB-mutated pheochromocytoma and paraganglioma. Cancers (Basel), 12(2), 280. https:\/\/doi.org\/10.3390\/cancers12020280","journal-title":"Cancers (Basel)"},{"key":"10009_CR128","unstructured":"ClinicalTrials.gov (2021). Current clinical trials on paraganglioma. https:\/\/clinicaltrials.gov\/ct2\/results?cond=paraganglioma&term=&cntry=&state=&city=&dist=. Accessed 26 Nov 2021."},{"key":"10009_CR129","unstructured":"ClinicalTrials.gov (2021). Clinical trials in pheochromocytoma. https:\/\/clinicaltrials.gov\/ct2\/results?cond=pheochromocytoma&term=&cntry=&state=&city=&dist=."},{"issue":"11","key":"10009_CR130","doi-asserted-by":"publisher","first-page":"2600","DOI":"10.1210\/en.2019-00410","volume":"160","author":"M Fankhauser","year":"2019","unstructured":"Fankhauser, M., Bechmann, N., Lauseker, M., Goncalves, J., Favier, J., Klink, B., et al. (2019). Synergistic highly potent targeted drug combinations in different pheochromocytoma models including human tumor cultures. Endocrinology, 160(11), 2600\u20132617. https:\/\/doi.org\/10.1210\/en.2019-00410","journal-title":"Endocrinology"},{"issue":"22","key":"10009_CR131","doi-asserted-by":"publisher","first-page":"2036","DOI":"10.1056\/NEJMoa2103425","volume":"385","author":"E Jonasch","year":"2021","unstructured":"Jonasch, E., Donskov, F., Iliopoulos, O., Rathmell, W. K., Narayan, V. K., Maughan, B. L., et al. (2021). Belzutifan for renal cell carcinoma in von Hippel-Lindau disease. New England Journal of Medicine, 385(22), 2036\u20132046. https:\/\/doi.org\/10.1056\/NEJMoa2103425","journal-title":"New England Journal of Medicine"},{"issue":"22","key":"10009_CR132","doi-asserted-by":"publisher","first-page":"2059","DOI":"10.1056\/NEJMoa2110051","volume":"385","author":"J Kamihara","year":"2021","unstructured":"Kamihara, J., Hamilton, K. V., Pollard, J. A., Clinton, C. M., Madden, J. A., Lin, J., et al. (2021). Belzutifan, a potent HIF2alpha inhibitor, in the Pacak-Zhuang syndrome. New England Journal of Medicine, 385(22), 2059\u20132065. https:\/\/doi.org\/10.1056\/NEJMoa2110051","journal-title":"New England Journal of Medicine"},{"issue":"4","key":"10009_CR133","doi-asserted-by":"publisher","first-page":"439","DOI":"10.1038\/onc.2016.225","volume":"36","author":"S Chouaib","year":"2017","unstructured":"Chouaib, S., Noman, M. Z., Kosmatopoulos, K., & Curran, M. A. (2017). Hypoxic stress: Obstacles and opportunities for innovative immunotherapy of cancer. Oncogene, 36(4), 439\u2013445. https:\/\/doi.org\/10.1038\/onc.2016.225","journal-title":"Oncogene"},{"key":"10009_CR134","doi-asserted-by":"publisher","first-page":"277","DOI":"10.3389\/fendo.2018.00277","volume":"9","author":"C Jimenez","year":"2018","unstructured":"Jimenez, C. (2018). Treatment for patients with malignant pheochromocytomas and paragangliomas: A perspective from the hallmarks of cancer. Front Endocrinol (Lausanne), 9, 277. https:\/\/doi.org\/10.3389\/fendo.2018.00277","journal-title":"Front Endocrinol (Lausanne)"},{"issue":"3","key":"10009_CR135","doi-asserted-by":"publisher","first-page":"e1290035","DOI":"10.1080\/2162402X.2017.1290035","volume":"6","author":"KP Fabian","year":"2017","unstructured":"Fabian, K. P., Chi-Sabins, N., Taylor, J. L., Fecek, R., Weinstein, A., & Storkus, W. J. (2017). Therapeutic efficacy of combined vaccination against tumor pericyte-associated antigens DLK1 and DLK2 in mice. Oncoimmunology, 6(3), e1290035. https:\/\/doi.org\/10.1080\/2162402X.2017.1290035","journal-title":"Oncoimmunology"},{"issue":"10","key":"10009_CR136","doi-asserted-by":"publisher","first-page":"1958","DOI":"10.1038\/mt.2013.133","volume":"21","author":"N Chi Sabins","year":"2013","unstructured":"Chi Sabins, N., Taylor, J. L., Fabian, K. P., Appleman, L. J., Maranchie, J. K., Stolz, D. B., et al. (2013). DLK1: A novel target for immunotherapeutic remodeling of the tumor blood vasculature. Molecular Therapy, 21(10), 1958\u20131968. https:\/\/doi.org\/10.1038\/mt.2013.133","journal-title":"Molecular Therapy"},{"issue":"5\u20136","key":"10009_CR137","doi-asserted-by":"publisher","first-page":"254","DOI":"10.1101\/gad.335109.119","volume":"34","author":"MS Cohen","year":"2020","unstructured":"Cohen, M. S. (2020). Interplay between compartmentalized NAD(+) synthesis and consumption: A focus on the PARP family. Genes & Development, 34(5\u20136), 254\u2013262. https:\/\/doi.org\/10.1101\/gad.335109.119","journal-title":"Genes & Development"},{"issue":"23","key":"10009_CR138","doi-asserted-by":"publisher","first-page":"4002","DOI":"10.1111\/febs.14297","volume":"284","author":"D Munnur","year":"2017","unstructured":"Munnur, D., & Ahel, I. (2017). Reversible mono-ADP-ribosylation of DNA breaks. FEBS Journal, 284(23), 4002\u20134016. https:\/\/doi.org\/10.1111\/febs.14297","journal-title":"FEBS Journal"},{"key":"10009_CR139","unstructured":"ClinicalTrials.gov (2021). Current clinical trials on themozolomide and olaparib in PPGL. https:\/\/clinicaltrials.gov\/ct2\/show\/NCT04394858?cond=pheochromocytoma&intr=olaparib&draw=2&rank=1. Accessed 26 Nov 2021."},{"key":"10009_CR140","doi-asserted-by":"publisher","first-page":"15422","DOI":"10.1038\/ncomms15422","volume":"8","author":"S Balani","year":"2017","unstructured":"Balani, S., Nguyen, L. V., & Eaves, C. J. (2017). Modeling the process of human tumorigenesis. Nature Communications, 8, 15422. https:\/\/doi.org\/10.1038\/ncomms15422","journal-title":"Nature Communications"},{"issue":"16","key":"10009_CR141","doi-asserted-by":"publisher","first-page":"3486","DOI":"10.1242\/dev.094011","volume":"140","author":"N Masoudi","year":"2013","unstructured":"Masoudi, N., Fancsalszky, L., Pourkarimi, E., Vellai, T., Alexa, A., Remenyi, A., et al. (2013). The NM23-H1\/H2 homolog NDK-1 is required for full activation of Ras signaling in C. elegans. Development, 140(16), 3486\u20133495. https:\/\/doi.org\/10.1242\/dev.094011","journal-title":"Development"},{"issue":"7584","key":"10009_CR142","doi-asserted-by":"publisher","first-page":"92","DOI":"10.1038\/nature16483","volume":"529","author":"R Kaletsky","year":"2016","unstructured":"Kaletsky, R., Lakhina, V., Arey, R., Williams, A., Landis, J., Ashraf, J., et al. (2016). The C. elegans adult neuronal IIS\/FOXO transcriptome reveals adult phenotype regulators. Nature, 529(7584), 92\u201396. https:\/\/doi.org\/10.1038\/nature16483","journal-title":"Nature"}],"container-title":["Cancer and Metastasis Reviews"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s10555-021-10009-z.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s10555-021-10009-z\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s10555-021-10009-z.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,9,15]],"date-time":"2024-09-15T06:04:35Z","timestamp":1726380275000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s10555-021-10009-z"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,12]]},"references-count":142,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2021,12]]}},"alternative-id":["10009"],"URL":"https:\/\/doi.org\/10.1007\/s10555-021-10009-z","relation":{},"ISSN":["0167-7659","1573-7233"],"issn-type":[{"value":"0167-7659","type":"print"},{"value":"1573-7233","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,12]]},"assertion":[{"value":"18 October 2021","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"4 December 2021","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"27 December 2021","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Not applicable (this is a review article).","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval"}},{"value":"Not applicable (this is a review article).","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Informed consent"}},{"value":"The authors declare no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}}]}}