ArticleEndocrine-related cancer2020
A xenograft and cell line model of SDH-deficient pheochromocytoma derived from Sdhb+/- rats.
Article in Endocrine-related cancer, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 17 papers.
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Who cites it
17 citing papers in PubMed, 35 citations in OpenAlex.
- Pilot study: transduction of primary paraganglioma chromaffin tumour cells with inducible c-MYC drives cell proliferation.Experimental and clinical endocrinology & diabetes : official journal, German Society of Endocrinology [and] German Diabetes Association · 2026Article
- Succinate dehydrogenase-deficient cancer cells have increased susceptibility to Ym155-induced DNA damage.Endocrine-related cancer · 2026Article
- Long-term culture of patient-derived pheochromocytoma organoids.Frontiers in endocrinology · 2026Article
- Mouse developmental defects, but not paraganglioma tumorigenesis, upon conditional Complex II loss in early Sox10FASEB bioAdvances · 2024Article
- Characterisation of an Adult Zebrafish Model forInternational journal of molecular sciences · 2024Article
- Three Dimensional Models of Endocrine Organs and Target Tissues Regulated by the Endocrine System.Cancers · 2023Review
- Preclinical Models of Neuroendocrine Neoplasia.Cancers · 2022Review
- Somatic Mutation Profiling in Head and Neck Paragangliomas.The Journal of clinical endocrinology and metabolism · 2022Article
- New Insights on the Genetics of Pheochromocytoma and Paraganglioma and Its Clinical Implications.Cancers · 2022Review
- Long-term in vitro 2D-culture of SDHB and SDHD-related human paragangliomas and pheochromocytomas.PloS one · 2022Article
- Model systems in SDHx-related pheochromocytoma/paraganglioma.Cancer metastasis reviews · 2021Review
- Article
- Unexpected obesity, rather than tumorigenesis, in a conditional mouse model of mitochondrial complex II deficiency.FASEB journal : official publication of the Federation of American Societies for Experimental Biology · 2021Article
- Mutation of the Cell Cycle Regulator p27kip1 Drives Pseudohypoxic Pheochromocytoma Development.Cancers · 2021Article
- Analysis of Colorectal Carcinogenesis Paradigm between Cold Constitution and Heat Constitution: Earlier ECM Collagen Deposition.Evidence-based complementary and alternative medicine : eCAM · 2021Article
- Pheochromocytoma/paraganglioma preclinical models: which to use and why?Endocrine connections · 2020Review
- Protective effects of SS-31 against SDHB suppression-mitochondrial dysfunction-EndMT axis-modulated CBT sclerosis and progression.American journal of translational research · 2020Article
Corrections and comments
- Erratum issued
Authors and funding
15 authors at 6 institutions in 2 countries.
Funding
Abstract
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, we developed a xenograft and cell line model of SDH-deficient pheochromocytoma from rats with a heterozygous germline Sdhb mutation. The genome, transcriptome and metabolome of this model, called RS0, closely resemble those of SDHB-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.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.