Animal and cell culture models of PPGLs - Achievements and limitations.
Horm. Metab. Res. 56, 51-64 (2024)
Research on rare tumors heavily relies on suitable models for basic and translational research. Paragangliomas (PPGL) are rare neuroendocrine tumors (NET), developing from adrenal (pheochromocytoma, PCC) or extra-adrenal (PGL) chromaffin cells, with an annual incidence of 2-8 cases per million. While most PPGL cases exhibit slow growth and are primarily treated with surgery, limited systemic treatment options are available for unresectable or metastatic tumors. Scarcity of appropriate models has hindered PPGL research, preventing the translation of omics knowledge into drug and therapy development. Human PPGL cell lines are not available, and few animal models accurately replicate the disease's genetic and phenotypic characteristics. This review provides an overview of laboratory models for PPGLs, spanning cellular, tissue, organ, and organism levels. We discuss their features, advantages, and potential contributions to diagnostics and therapeutics. Interestingly, it appears that in the PPGL field, disease models already successfully implemented in other cancers have not been fully explored.
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Publikationstyp
Artikel: Journalartikel
Dokumenttyp
Review
Typ der Hochschulschrift
Herausgeber
Schlagwörter
pheochromocytoma; paraganglioma; PDX; SDHx; neuroendocrine tumor; Metastatic Pheochromocytoma; Molecular Characterization; Paraganglioma Xenografts; Transcriptome Analysis; Signaling Pathways; Adrenal-gland; Mouse Model; Growth-rate; In-vivo; Sdhb
Keywords plus
Sprache
englisch
Veröffentlichungsjahr
2024
Prepublished im Jahr
0
HGF-Berichtsjahr
2024
ISSN (print) / ISBN
0018-5043
e-ISSN
1439-4286
ISBN
Bandtitel
Konferenztitel
Konferzenzdatum
Konferenzort
Konferenzband
Quellenangaben
Band: 56,
Heft: 1,
Seiten: 51-64
Artikelnummer: ,
Supplement: ,
Reihe
Verlag
Thieme
Verlagsort
Rudigerstr 14, D-70469 Stuttgart, Germany
Tag d. mündl. Prüfung
0000-00-00
Betreuer
Gutachter
Prüfer
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0000-00-00
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0000-00-00
Anmelder/Inhaber
weitere Inhaber
Anmeldeland
Priorität
Begutachtungsstatus
Peer reviewed
POF Topic(s)
30201 - Metabolic Health
90000 - German Center for Diabetes Research
Forschungsfeld(er)
Helmholtz Diabetes Center
PSP-Element(e)
G-502590-001
G-501900-251
Förderungen
Deutsche Forschungsgemeinschaft (CRC/Transregio 205 The Adrenal: Central Relay in Health and Disease)
Copyright
Erfassungsdatum
2024-01-07