PMID- 9676841
OWN - NLM
STAT- MEDLINE
DCOM- 19980916
LR  - 20041117
IS  - 1078-0432 (Print)
IS  - 1078-0432 (Linking)
VI  - 4
IP  - 7
DP  - 1998 Jul
TI  - 11q13 allelic loss in pituitary tumors in patients with multiple endocrine 
      neoplasia syndrome type 1.
PG  - 1673-8
AB  - Pituitary adenomas may develop sporadically or as part of the multiple endocrine 
      neoplasia type 1 (MEN 1) syndrome. The gene responsible for MEN 1 syndrome was 
      recently identified and cloned. Low rates of MEN 1 gene mutations and deletions 
      have been reported in sporadic pituitary adenomas. To elucidate the role of the 
      MEN 1 gene in the pathogenesis of MEN 1-associated pituitary tumors, we examined 
      pituitary adenomas from 11 MEN 1 patients for the presence of 11q13 allelic loss. 
      Ten of the 11 pituitary tumors were informative by PCR-based loss of 
      heterozygosity analysis. Using a combination of family pedigree analysis and 
      restriction analysis directed at the mutated allele in 8 of the 10 informative 
      cases, it was demonstrated in all 8 cases that it is the wild-type allele that 
      undergoes deletion. All 11 tumors, 4 of which were growth hormone secreting, were 
      additionally analyzed for mutation in the Gs alpha subunit (gsp) gene. None of 
      the tumors (0 of 11 tumors) revealed a gsp gene mutation. Therefore, genetic 
      alterations of the MEN 1 gene seem to play a dominant role in MEN 1-associated 
      pituitary tumorigenesis, whereas gsp gene mutations do not seem to be a frequent 
      event in either growth hormone-secreting or other types of MEN 1-associated 
      pituitary tumors. These results suggest that MEN 1-associated pituitary tumors 
      develop via genetic pathways that differ from those of most sporadic pituitary 
      tumors.
FAU - Weil, R J
AU  - Weil RJ
AD  - Surgical Neurology Branch, National Institute of Neurological Disorders and 
      Stroke, NIH, Bethesda, Maryland 20892, USA. rweil@box-r.nih.gov
FAU - Vortmeyer, A O
AU  - Vortmeyer AO
FAU - Huang, S
AU  - Huang S
FAU - Boni, R
AU  - Boni R
FAU - Lubensky, I A
AU  - Lubensky IA
FAU - Pack, S
AU  - Pack S
FAU - Marx, S J
AU  - Marx SJ
FAU - Zhuang, Z
AU  - Zhuang Z
FAU - Oldfield, E H
AU  - Oldfield EH
LA  - eng
PT  - Journal Article
PL  - United States
TA  - Clin Cancer Res
JT  - Clinical cancer research : an official journal of the American Association for 
      Cancer Research
JID - 9502500
SB  - IM
MH  - Adenoma/*genetics
MH  - Adult
MH  - Aged
MH  - *Alleles
MH  - Child, Preschool
MH  - Female
MH  - *Gene Deletion
MH  - Humans
MH  - Loss of Heterozygosity
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/*genetics
MH  - Pituitary Neoplasms/*genetics
EDAT- 1998/07/24 00:00
MHDA- 1998/07/24 00:01
CRDT- 1998/07/24 00:00
PHST- 1998/07/24 00:00 [pubmed]
PHST- 1998/07/24 00:01 [medline]
PHST- 1998/07/24 00:00 [entrez]
PST - ppublish
SO  - Clin Cancer Res. 1998 Jul;4(7):1673-8.