Endocrine Abstracts

Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disease characterised by the combined occurrence of parathyroid, pancreatic islet and anterior pituitary tumours. Over 60% of the anterior pituitary tumours in MEN1 patients are prolactinomas, ~25% are somatotrophinomas, ~5% are corticotrophinomas, and the remainder appear to be non-functioning. The MEN1 gene, a tumour suppressor, is located on chromosome 11q13, and more than 1300 MEN1 mutations, which are lik...

The pituitary is highly sensitive to cell cycle disruptions. Although CDK gene mutations have not readily been identified in human pituitary tumors, overexpression of cyclins and dysregulation of CDK inhibitors are encountered in pituitary adenomas, indicating the importance of CDK activation for potential therapeutic targeting. However, it is difficult to predict which CDK inhibitor(s) may be effective against particular tumor types in vivo. Therefore, animal models fa...

Activating mutations in the gene encoding β-catenin have been identified in the paediatric form of human craniopharyngioma (adamantinomatous craniopharyngioma, ACP), an aggressive pituitary tumour accounting for up to 10% of paediatric intracranial tumours. Recently, we generated an ACP mouse model and revealed that, as in human ACP, nucleocytoplasmic accumulation of β-catenin (βcat-nc) and over-activation of the Wnt/β-catenin pathway occurs only in very fe...

Pituitary adenomas are commonly occurring lesions, only some of which will result in clinically significant disease. The underlying molecular changes leading to monoclonal cell proliferation with self-limited growth in the majority of cases, and continuous growth in a small proportion, are currently unknown. While hormonal influences are clearly important additional factors, the primary abnormality will ultimately lead to up-regulation of the cell cycle machinery. Hormonal fee...