ArticleAmerican journal of physiology. Endocrinology and metabolism2008
Differential expression of the regulated catecholamine secretory pathway in different hereditary forms of pheochromocytoma.
Article in American journal of physiology. Endocrinology and metabolism, 2008. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 34 papers, 1 of them a synthesis that pooled it.
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Who cites it
34 citing papers in PubMed, 1 synthesis or guideline pooled it, 84 citations in OpenAlex.
- Silent pheochromocytoma and paraganglioma: Systematic review and proposed definitions for standardized terminology.Frontiers in endocrinology · 2022Pooled it
- Quantitative [EJNMMI reports · 2025Article
- [European journal of nuclear medicine and molecular imaging · 2025Article
- Cellular mechanisms of hormone secretion in neuroendocrine tumors: what goes wrong?Frontiers in cell and developmental biology · 2025Review
- Management of Pheochromocytomas and Paragangliomas.The Surgical clinics of North America · 2024Review
- Prevention and management of hypertensive crises in children with pheochromocytoma and paraganglioma.Frontiers in endocrinology · 2024Review
- Influence of duration of preoperative treatment with phenoxybenzamine and secretory phenotypes on perioperative hemodynamics and postoperative outcomes in pheochromocytoma and paraganglioma.Frontiers in endocrinology · 2023Article
- Pheochromocytoma/paraganglioma-associated cardiomyopathy.Frontiers in endocrinology · 2023Review
- Nomogram for predicting hemodynamic instability and the association between preoperative 3D printing and hemodynamic instability during laparoscopic adrenalectomy for pheochromocytoma.American journal of cancer research · 2023Article
- Impact of body composition and genotype on haemodynamics during surgery for pheochromocytoma and paraganglioma.Journal of cachexia, sarcopenia and muscle · 2022Article
- Personalized Management of Pheochromocytoma and Paraganglioma.Endocrine reviews · 2022Review
- Treatment of Pheochromocytoma Cells with Recurrent Cycles of Hypoxia: A New Pseudohypoxic In Vitro Model.Cells · 2022Article
- Metanephrine and normetanephrine associated with subclinical myocardial injuries in pheochromocytoma and paraganglioma.Frontiers in oncology · 2022Article
- Pediatric Metastatic Pheochromocytoma and Paraganglioma: Clinical Presentation and Diagnosis, Genetics, and Therapeutic Approaches.Frontiers in endocrinology · 2022Review
- Acute cardiac complications and subclinical myocardial injuries associated with pheochromocytoma and paraganglioma.BMC cardiovascular disorders · 2021Article
- Metastatic pheochromocytoma and paraganglioma: signs and symptoms related to catecholamine secretion.Discover oncology · 2021Article
- Mutation of the Cell Cycle Regulator p27kip1 Drives Pseudohypoxic Pheochromocytoma Development.Cancers · 2021Article
- Pathophysiology and Acute Management of Tachyarrhythmias in Pheochromocytoma: JACC Review Topic of the Week.Journal of the American College of Cardiology · 2020Review
- Review
- Pheochromocytoma and paraganglioma: genotype versus anatomic location as determinants of tumor phenotype.Cell and tissue research · 2018Review
Corrections and comments
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Authors and funding
13 authors at 1 institution in 1 country.
Funding
Abstract
Pheochromocytomas in patients with von Hippel-Lindau (VHL) syndrome and multiple endocrine neoplasia type 2 (MEN 2) differ in the types and amounts of catecholamines produced and the resulting signs and symptoms. We hypothesized the presence of different processes of catecholamine release reflecting differential expression of components of the regulated secretory pathway among the two types of hereditary tumors. Differences in catecholamine secretion from tumors in patients with VHL syndrome (n = 47) and MEN 2 (n = 32) were examined using measurements of catecholamines in tumor tissue, urine, and plasma, the last of which was under baseline conditions in all subjects and in a subgroup of patients who received intravenous glucagon to provoke catecholamine release. Microarray and proteomics analyses, quantitative PCR, and Western blotting were used to assess expression of tumor tissue secretory pathway components. The rate constant for baseline catecholamine secretion was 20-fold higher in VHL than in MEN 2 tumors (0.359 +/- 0.094 vs. 0.018 +/- 0.009 day(-1)), but catecholamine release was responsive only to glucagon in MEN 2 tumors. Compared with tumors from MEN 2 patients, those from VHL patients were characterized by reduced expression of numerous components of the regulated secretory pathway (e.g., SNAP25, syntaxin, rabphilin 3A, annexin A7, calcium-dependent secretion activator). The mutation-dependent differences in expression of secretory pathway components indicate a more mature regulated secretory pathway in MEN 2 than VHL tumors. These data provide a unique mechanistic link to explain how variations in the molecular machinery governing exocytosis may contribute to clinical differences in the secretion of neurotransmitters or hormones and the subsequent presentation of a disease.
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