Pseudohypoxic tumors activate pro-oncogenic pathways typically associated with severe hypoxia even when sufficient oxygen is present, leading to highly aggressive tumors. Prime examples are pseudohypoxic pheochromocytomas and paragangliomas (p-PPGLs), neuroendendocrine tumors currently lacking effective therapy. Previous attempts to generate mouse models for p-PPGLs all failed. Here, we describe that the rat MENX line, carrying a Cdkn1b (p27) frameshift-mutation, spontaneously develops pseudohypoxic pheochromocytoma (p-PCC).

Mutation of the Cell Cycle Regulator p27kip1 Drives Pseudohypoxic Pheochromocytoma Development

Pellegata, Natalia S
2021-01-01

Abstract

Pseudohypoxic tumors activate pro-oncogenic pathways typically associated with severe hypoxia even when sufficient oxygen is present, leading to highly aggressive tumors. Prime examples are pseudohypoxic pheochromocytomas and paragangliomas (p-PPGLs), neuroendendocrine tumors currently lacking effective therapy. Previous attempts to generate mouse models for p-PPGLs all failed. Here, we describe that the rat MENX line, carrying a Cdkn1b (p27) frameshift-mutation, spontaneously develops pseudohypoxic pheochromocytoma (p-PCC).
2021
Oncogenesis & Cancer Research
Inglese
13
1
126
2-hydroxyglutarate; 5-hmC; MENX; PPGLs; angiogenesis; animal model; cell cycle; endogenous pheochromocytoma; hypermethylation; mitochondrial dysfunction; oncometabolites; pseudohypoxia
10
info:eu-repo/semantics/article
262
Mohr, Hermine; Ballke, Simone; Bechmann, Nicole; Gulde, Sebastian; Malekzadeh-Najafabadi, Jaber; Peitzsch, Mirko; Ntziachristos, Vasilis; Steiger, Kat...espandi
1 Contributo su Rivista::1.1 Articolo in rivista
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11571/1372735
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