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MEN1 gene analysis in sporadic adrenocortical neoplasms.
| Content Provider | Semantic Scholar |
|---|---|
| Author | Heppner, Christina Reincke, Martin Agarwal, Sunita Mora, Puig Allolio, Bruno Burns, A. Lee Spiegel, Allen M. Marx, Stephen J. |
| Copyright Year | 1999 |
| Abstract | Adrenocortical tumors occur as sporadic tumors, as part of the multiple endocrine neoplasia type 1 (MEN1) syndrome or as part of other hereditary disorders. We recently cloned the MEN1 gene, a tumor-suppressor gene located on chromosome 11q13. Subsequently, we showed that sequential somatic inactivation of both alleles of the MEN1 gene contributes to the development of some sporadic endocrine neoplasms (parathyroid, enteropancreatic neuroendocrine, bronchial carcinoid, and pituitary tumors). We now studied whether somatic inactivation of the MEN1 gene contributes to the pathogenesis of sporadic adrenocortical neoplasms. Seven adrenocortical carcinomas, 2 adrenocortical carcinoma cell lines, and 11 aldosterone-secreting, 8 cortisol-secreting, and 5 nonsecreting benign adrenocortical tumors were studied. Seven tumors (5 of 5 carcinomas, 2 of 21 nonsecreting benign adenomas; P < 0.001) exhibited loss of heterozygosity on 11q13. All 33 tumors and cell lines were screened for mutation throughout the MEN1 open-reading frame and adjacent splice junctions. None exhibited a mutation within the MEN1-coding region. We conclude that somatic MEN1 mutation within the MEN1-coding region does not occur commonly in sporadic adrenocortical tumors, although the majority of adrenocortical carcinomas exhibit 11q13 loss of heterozygosity. |
| Starting Page | 92 |
| Ending Page | 100 |
| Page Count | 9 |
| File Format | PDF HTM / HTML |
| Alternate Webpage(s) | http://jcem.endojournals.org/content/84/1/216.full.pdf |
| PubMed reference number | 9920087v1 |
| Volume Number | 84 |
| Issue Number | 1 |
| Journal | The Journal of clinical endocrinology and metabolism |
| Language | English |
| Access Restriction | Open |
| Subject Keyword | 11q13 Adrenocortical carcinoma Aldosterone Cell secretion Diploid Cell Endocrine Gland Neoplasms Genes, vif Hereditary Diseases Heterozygote Hydrocortisone Loss of Heterozygosity Multiple Endocrine Neoplasia Type 1 Mutation Neurosecretory Systems Ovarian Large Cell Neuroendocrine Carcinoma Pituitary Neoplasms Pulmonary carcinoid tumor Reading Frames (Nucleotide Sequence) |
| Content Type | Text |
| Resource Type | Article |
