Characterization of MENX-associated pituitary tumours

Marinoni, Ilaria; Lee, Misu; Mountford, Steffeni; Perren, Aurel; Bravi, Ilaria; Jennen, Louise; Feuchtinger, Annette; Drouin, Jacques; Roncaroli, Federico; Pellegata, Natalia S (2012). Characterization of MENX-associated pituitary tumours. Neuropathology & applied neurobiology, 39(3), pp. 256-269. Oxford: Blackwell Scientific Publications 10.1111/j.1365-2990.2012.01278.x

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Aims: The aim of this study is to evaluate the pathological features, serum hormone levels and ex-vivo cultures of pituitary adenomas that occur in rats affected by MENX syndrome. MENX is multiple endocrine neoplasia syndrome caused by a germline mutation in the cell cycle inhibitor p27. Characterisation of MENX adenomas is a prerequisite to exploit this animal model for molecular and translational studies of pituitary adenomas. Methods: We investigated MENX pituitary adenomas with immunohistochemistry, double immunofluorescence, electron microscopy, RT-PCR, measurement of serum hormone levels and ex-vivo cultures. Results: Adenomas in MENX rats belong to the gonadotroph lineage. They start from 4 months of age as multiple neoplastic nodules and progress to become large lesions that efface the gland. Adenomas are composed of chromophobic cells predominantly expressing the glycoprotein alpha-subunit (αGSU). They show mitotic activity and high Ki67 labelling. A few neoplastic cells co-express gonadotrophins and the transcription factor SF1, together with growth hormone or prolactin and Pit-1, suggesting that they are not fully committed to one cell lineage. Ex vivo cultures show features similar to the primary tumour. Conclusions: Our results suggest that p27 function is critical in regulating gonadotroph cells growth. The MENX syndrome represents a unique model to elucidate the physiological and molecular mechanisms mediating the pathogenesis of gonadotroph adenomas. © 2012 The Authors. Neuropathology and Applied Neurobiology © 2012 British Neuropathological Society.

Item Type:

Journal Article (Original Article)

Division/Institute:

04 Faculty of Medicine > Service Sector > Institute of Pathology

UniBE Contributor:

Marinoni, Ilaria and Perren, Aurel

Subjects:

500 Science > 570 Life sciences; biology
600 Technology > 610 Medicine & health

ISSN:

0305-1846

Publisher:

Blackwell Scientific Publications

Language:

English

Submitter:

Factscience Import

Date Deposited:

04 Oct 2013 14:32

Last Modified:

19 Jun 2014 07:45

Publisher DOI:

10.1111/j.1365-2990.2012.01278.x

PubMed ID:

22524684

Web of Science ID:

000316188800005

URI:

https://boris.unibe.ch/id/eprint/12455 (FactScience: 218799)

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