{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T12:49:51Z","timestamp":1790254191656,"version":"4.1.0"},"reference-count":0,"publisher":"Bioscientifica","issue":"6","content-domain":{"domain":["erc.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2020,6]]},"abstract":"<jats:p>\n                    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\n                    <jats:italic>SDHB<\/jats:italic>\n                    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, we developed a xenograft and cell line model of SDH-deficient pheochromocytoma from rats with a heterozygous germline\n                    <jats:italic>Sdhb<\/jats:italic>\n                    mutation. The genome, transcriptome and metabolome of this model, called RS0, closely resemble those of\n                    <jats:italic>SDHB<\/jats:italic>\n                    -mutated human PCPGs, making it the most valid model now available. Strategies employed to develop RS0 may be broadly applicable to other SDH-deficient tumors.\n                  <\/jats:p>","DOI":"10.1530\/erc-19-0474","type":"journal-article","created":{"date-parts":[[2020,4,6]],"date-time":"2020-04-06T08:27:56Z","timestamp":1586161676000},"page":"337-354","update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-1","source":"Crossref","is-referenced-by-count":32,"title":["A xenograft and cell line model of SDH-deficient pheochromocytoma derived from Sdhb+\/\u2212 rats"],"prefix":"10.1530","volume":"27","author":[{"given":"James F","family":"Powers","sequence":"first","affiliation":[{"name":"1Department of Pathology and Laboratory Medicine, Tufts Medical Center, Tufts University School of Medicine, Boston, Massachusetts, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brent","family":"Cochran","sequence":"additional","affiliation":[{"name":"2Department of Developmental, Molecular and Chemical Biology, Tufts University School of Medicine, Boston, Massachusetts, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James D","family":"Baleja","sequence":"additional","affiliation":[{"name":"2Department of Developmental, Molecular and Chemical Biology, Tufts University School of Medicine, Boston, Massachusetts, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hadley D","family":"Sikes","sequence":"additional","affiliation":[{"name":"3Department of Chemical Engineering, Massachusetts Institute of Technology, Cambridge, Massachusetts, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew D","family":"Pattison","sequence":"additional","affiliation":[{"name":"4Department of Clinical Pathology, University of Melbourne, Melbourne, Victoria, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xue","family":"Zhang","sequence":"additional","affiliation":[{"name":"2Department of Developmental, Molecular and Chemical Biology, Tufts University School of Medicine, Boston, Massachusetts, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Inna","family":"Lomakin","sequence":"additional","affiliation":[{"name":"1Department of Pathology and Laboratory Medicine, Tufts Medical Center, Tufts University School of Medicine, Boston, Massachusetts, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annette","family":"Shepard-Barry","sequence":"additional","affiliation":[{"name":"1Department of Pathology and Laboratory Medicine, Tufts Medical Center, Tufts University School of Medicine, Boston, Massachusetts, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karel","family":"Pacak","sequence":"additional","affiliation":[{"name":"5Section on Medical Neuroendocrinology, Eunice Kennedy Shriver Division National Institute of Child Health and Human Development, Bethesda, Maryland, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sun Jin","family":"Moon","sequence":"additional","affiliation":[{"name":"3Department of Chemical Engineering, Massachusetts Institute of Technology, Cambridge, Massachusetts, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Troy F","family":"Langford","sequence":"additional","affiliation":[{"name":"3Department of Chemical Engineering, Massachusetts Institute of Technology, Cambridge, Massachusetts, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kassi Taylor","family":"Stein","sequence":"additional","affiliation":[{"name":"3Department of Chemical Engineering, Massachusetts Institute of Technology, Cambridge, Massachusetts, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard W","family":"Tothill","sequence":"additional","affiliation":[{"name":"4Department of Clinical Pathology, University of Melbourne, Melbourne, Victoria, Australia"},{"name":"6Peter MacCallum Cancer Centre, Melbourne, Victoria, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yingbin","family":"Ouyang","sequence":"additional","affiliation":[{"name":"7Cyagen US Inc, Santa Clara, California, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arthur S","family":"Tischler","sequence":"additional","affiliation":[{"name":"1Department of Pathology and Laboratory Medicine, Tufts Medical Center, Tufts University School of Medicine, Boston, Massachusetts, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"416","container-title":["Endocrine-Related Cancer"],"original-title":[],"link":[{"URL":"https:\/\/erc.bioscientifica.com\/view\/journals\/erc\/27\/6\/ERC-19-0474.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/erc.bioscientifica.com\/downloadpdf\/journals\/erc\/27\/6\/ERC-19-0474.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,20]],"date-time":"2026-07-20T13:55:33Z","timestamp":1784555733000},"score":1,"resource":{"primary":{"URL":"http:\/\/journals.bioscientifica.com\/erc\/article\/27\/6\/337-354\/25503"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,6]]},"references-count":0,"journal-issue":{"issue":"6"},"URL":"https:\/\/doi.org\/10.1530\/erc-19-0474","relation":{},"ISSN":["1351-0088","1479-6821"],"issn-type":[{"value":"1351-0088","type":"print"},{"value":"1479-6821","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,6]]}}}