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Sigala, S.* ; Bothou, C.* ; Penton, D.* ; Abate, A.* ; Peitzsch, M.* ; Cosentini, D.* ; Tiberio, G.A.M.* ; Bornstein, S.R. ; Berruti, A.* ; Hantel, C.*

A comprehensive investigation of steroidogenic signaling in classical and new experimental cell models of adrenocortical carcinoma.

Cells 11:1439 (2022)
Publ. Version/Full Text Research data DOI PMC
Open Access Gold
Creative Commons Lizenzvertrag
Adrenocortical carcinoma is a heterogeneous and aggressive cancer that originates from steroidogenic cells within the adrenal cortex. In this study, we have assessed for the preclinical gold standard NCI-H295 in direct comparison with the more recently established MUC-1 and a here newly reported ACC cell line (TVBF-7) the mutational status of important driver genes (TP53, MEN1, PRKAR1A, CTNNB1, APC, ZNRF-3, IGF-2, EGFR, RB1, BRCA1, BRCA2, RET, GNAS and PTEN), Wnt-signaling specificities (CTNNB1 mutation vs. APC mutation vs. wildtype), steroidogenic-(CYP11A1, CYP17A1, HSD3B2, HSD17B4, CYP21A2, CYP11B1, CYP11B2, MC2R, AT1R) and nuclear-receptor-signaling (AR, ER, GCR), varying electrophysiological potentials as well as highly individual hormone secretion profiles (Cortisol, Aldosterone, DHEA, DHEAS, Testosterone, 17-OH Progesterone, among others) which were investigated under basal and stimulated conditions (ACTH, AngII, FSK). Our findings reveal important genetic and pathophysiological characteristics for these three cell lines and reveal the importance of such cell-line panels reflecting differential endocrine functionalities to thereby better reflect clinically well-known ACC patient heterogeneities in preclinical studies.
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Publication type Article: Journal article
Document type Scientific Article
Corresponding Author
Keywords Adrenocortical Carcinoma Cell Lines ; Electrophysiology ; Genotype ; Muc-1 ; Nci-h295 ; Steroidogenesis ; Tvbf-7
ISSN (print) / ISBN 2073-4409
e-ISSN 2073-4409
Journal Cells
Quellenangaben Volume: 11, Issue: 9, Pages: , Article Number: 1439 Supplement: ,
Publisher MDPI
Publishing Place Basel
Non-patent literature Publications
Institute(s) Institute of Pancreatic Islet Research (IPI)
Grants Italian Association for Cancer Research
Heuberg Foundation
Uniscientia Foundation