PMID- 9920087
OWN - NLM
STAT- MEDLINE
DCOM- 19990203
LR  - 20061115
IS  - 0021-972X (Print)
IS  - 0021-972X (Linking)
VI  - 84
IP  - 1
DP  - 1999 Jan
TI  - MEN1 gene analysis in sporadic adrenocortical neoplasms.
PG  - 216-9
AB  - 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.
FAU - Heppner, C
AU  - Heppner C
AD  - Metabolic Diseases Branch, National Institute of Diabetes and Digestive and 
      Kidney Diseases, National Institutes of Health, Bethesda, Maryland 20892-1802, 
      USA. christinah@bdg10.niddk.nih.gov
FAU - Reincke, M
AU  - Reincke M
FAU - Agarwal, S K
AU  - Agarwal SK
FAU - Mora, P
AU  - Mora P
FAU - Allolio, B
AU  - Allolio B
FAU - Burns, A L
AU  - Burns AL
FAU - Spiegel, A M
AU  - Spiegel AM
FAU - Marx, S J
AU  - Marx SJ
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - J Clin Endocrinol Metab
JT  - The Journal of clinical endocrinology and metabolism
JID - 0375362
RN  - 0 (MEN1 protein, human)
RN  - 0 (Neoplasm Proteins)
RN  - 0 (Proto-Oncogene Proteins)
SB  - IM
MH  - Adrenal Cortex Neoplasms/*genetics
MH  - Chromosomes, Human, Pair 11
MH  - Humans
MH  - Loss of Heterozygosity
MH  - Multiple Endocrine Neoplasia Type 1/*genetics
MH  - Mutation
MH  - Neoplasm Proteins/*genetics
MH  - *Proto-Oncogene Proteins
EDAT- 1999/01/27 00:00
MHDA- 1999/01/27 00:01
CRDT- 1999/01/27 00:00
PHST- 1999/01/27 00:00 [pubmed]
PHST- 1999/01/27 00:01 [medline]
PHST- 1999/01/27 00:00 [entrez]
AID - 10.1210/jcem.84.1.5388 [doi]
PST - ppublish
SO  - J Clin Endocrinol Metab. 1999 Jan;84(1):216-9. doi: 10.1210/jcem.84.1.5388.