ArticleThe Journal of clinical endocrinology and metabolism2015
Genotype-specific differences in the tumor metabolite profile of pheochromocytoma and paraganglioma using untargeted and targeted metabolomics.
Article in The Journal of clinical endocrinology and metabolism, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers.
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Who cites it
23 citing papers in PubMed, 37 citations in OpenAlex.
- [Germline mutations define the prognosis and therapy of pheochromocytomas and paragangliomas].Magyar onkologia · 2025Review
- Characterisation of an Adult Zebrafish Model forInternational journal of molecular sciences · 2024Article
- Cancer predisposing syndromes in childhood and adolescence pose several challenges necessitating interdisciplinary care in dedicated programs.Frontiers in pediatrics · 2024Article
- Metabolomics in paraganglioma: applications and perspectives from genetics to therapy.Endocrine-related cancer · 2023Article
- SDHx mutation and pituitary adenoma: can in vivo 1H-MR spectroscopy unravel the link?Endocrine-related cancer · 2023Article
- Hereditary Endocrine Tumor Registries.Journal of the Endocrine Society · 2023Article
- Metabolomics-A Tool to Find Metabolism of Endocrine Cancer.Metabolites · 2022Review
- Single-nuclei and bulk-tissue gene-expression analysis of pheochromocytoma and paraganglioma links disease subtypes with tumor microenvironment.Nature communications · 2022Article
- Genetics of Pheochromocytomas and Paragangliomas Determine the Therapeutical Approach.International journal of molecular sciences · 2022Review
- Loss of SDHB Promotes Dysregulated Iron Homeostasis, Oxidative Stress, and Sensitivity to Ascorbate.Cancer research · 2021Article
- Metabolic Regulation of Epithelial to Mesenchymal Transition: Implications for Endocrine Cancer.Frontiers in endocrinology · 2019Review
- New insights on the pathogenesis of paraganglioma and pheochromocytoma.F1000Research · 2018Review
- Genomic Landscape of Pheochromocytoma and Paraganglioma.Trends in cancer · 2018Review
- Mitochondrial determinants of cancer health disparities.Seminars in cancer biology · 2017Review
- New Insights into the Nuclear Imaging Phenotypes of Cluster 1 Pheochromocytoma and Paraganglioma.Trends in endocrinology and metabolism: TEM · 2017Review
- The Evolving Role of Succinate in Tumor Metabolism: AnJournal of nuclear medicine : official publication, Society of Nuclear Medicine · 2017Article
- Metabologenomics of Phaeochromocytoma and Paraganglioma: An Integrated Approach for Personalised Biochemical and Genetic Testing.The Clinical biochemist. Reviews · 2017Review
- Metabolomic Biomarkers in Urine of Cushing's Syndrome Patients.International journal of molecular sciences · 2017Article
- Article
- Mapping Extracellular pH of Gliomas in Presence of Superparamagnetic Nanoparticles: Towards Imaging the Distribution of Drug-Containing Nanoparticles and Their Curative Effect on the Tumor Microenvironment.Contrast media & molecular imaging · 2017Article
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Authors and funding
14 authors at 5 institutions in 3 countries.
Funding
Abstract
CONTEXT AND
objectivePheochromocytomas and paragangliomas (PGLs) are neuroendocrine tumors of sympathetic or parasympathetic paraganglia. Nearly 40% of PGLs are caused by germline mutations. The present study investigated the effect of genetic alterations on metabolic networks in PGLs.
designHomogenates of 32 sporadic PGLs and 48 PGLs from patients with mutations in SDHB, SDHD, SDHAF-2, VHL, RET, and NF-1 were subjected to proton ((1)H) nuclear magnetic resonance (NMR) spectroscopy at 500 MHz for untargeted and HPLC tandem mass spectrometry for targeted metabolite profiling.
results(1)H NMR spectroscopy identified 28 metabolites in PGLs of which 12 showed genotype-specific differences. Part of these results published earlier reported low complex II activity (P < .0001) and low ATP/ADP/AMP content (P < .001) in SDH-related PGLs compared with sporadics and PGLs of other genotypes. Extending these results, low levels of N-acetylaspartic acid (NAA; P < .05) in SDH tumors and creatine (P < .05) in VHL tumors were observed compared with sporadics and other genotypes. Positive correlation was observed between NAA and ATP/ADP/AMP content (P < .001) and NAA and complex II activity (P < .0001) of PGLs. Targeted purine analysis in PGLs showed low adenine in cluster 1 compared with cluster 2 tumors (SDH P < .0001; VHL P < .05) whereas lower levels (P < .05) of guanosine and hypoxanthine were observed in RET tumors compared with SDH tumors. Principal component analysis (PCA) of metabolites could distinguish PGLs of different genotypes.
conclusionsThe present study gives a comprehensive picture of alterations in energy metabolism in SDH- and VHL-related PGLs and establishes the interrelationship of energy metabolism and amino acid and purine metabolism in PGLs.
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