PMID- 2568586
OWN - NLM
STAT- MEDLINE
DCOM- 19890816
LR  - 20041117
IS  - 0028-4793 (Print)
IS  - 0028-4793 (Linking)
VI  - 321
IP  - 4
DP  - 1989 Jul 27
TI  - Clonality of parathyroid tumors in familial multiple endocrine neoplasia type 1.
PG  - 213-8
AB  - Familial multiple endocrine neoplasia type 1 (MEN-1) is characterized by tumors 
      of the parathyroids, endocrine pancreas, and anterior pituitary. Since the gene 
      associated with MEN-1, located on chromosome 11 (11q13), may normally inhibit 
      tumor proliferation, tumors could arise from inactivation of one or both of the 
      alleles. However, parathyroid tumors in patients with MEN-1 have been considered 
      to result from polyclonal hyperplasia. Using genetic probes, we tested 
      parathyroid tumors for a monoclonal component, represented by a loss of alleles 
      at any of eight loci along chromosome 11. Ten of 16 tumors from 14 patients with 
      familial MEN-1 had losses of alleles from chromosome 11. Tumors with losses were 
      larger than those without (1.6 vs. 0.2 g; P less than 0.002), suggesting that a 
      monoclonal adenoma may develop after a phase of polyclonal hyperplasia. In 7 of 
      10 tumors, the subregion of loss was less than the full length of chromosome 11 
      but always included one copy of the MEN-1 locus. Of 34 sporadic adenomas from 
      patients without MEN-1, 9 showed similar allelic losses in chromosome 11; in 7 
      the losses included the apparent MEN-1 locus. We conclude that many 
      "hyperplastic" parathyroid tumors in familial MEN-1 are in fact monoclonal and 
      may progress or even begin to develop by inactivation of the MEN-1 gene (at 
      11q13) in a precursor cell. Some sporadic adenomas have allelic losses on 
      chromosome 11, which may also involve the MEN-1 gene.
FAU - Friedman, E
AU  - Friedman E
AD  - Molecular Pathophysiology Branch, National Institute of Diabetes and Digestive 
      and Kidney Diseases, Bethesda, Md.
FAU - Sakaguchi, K
AU  - Sakaguchi K
FAU - Bale, A E
AU  - Bale AE
FAU - Falchetti, A
AU  - Falchetti A
FAU - Streeten, E
AU  - Streeten E
FAU - Zimering, M B
AU  - Zimering MB
FAU - Weinstein, L S
AU  - Weinstein LS
FAU - McBride, W O
AU  - McBride WO
FAU - Nakamura, Y
AU  - Nakamura Y
FAU - Brandi, M L
AU  - Brandi ML
AU  - et al.
LA  - eng
PT  - Journal Article
PL  - United States
TA  - N Engl J Med
JT  - The New England journal of medicine
JID - 0255562
SB  - IM
EIN - N Engl J Med 1989 Oct 12;321(15):1057
MH  - Adenoma/*genetics
MH  - Alleles
MH  - *Chromosome Deletion
MH  - *Chromosomes, Human, Pair 11
MH  - Clone Cells
MH  - Humans
MH  - Hyperplasia
MH  - Multiple Endocrine Neoplasia/*genetics/pathology
MH  - Parathyroid Neoplasms/*genetics/pathology
EDAT- 1989/07/27 00:00
MHDA- 1989/07/27 00:01
CRDT- 1989/07/27 00:00
PHST- 1989/07/27 00:00 [pubmed]
PHST- 1989/07/27 00:01 [medline]
PHST- 1989/07/27 00:00 [entrez]
AID - 10.1056/NEJM198907273210402 [doi]
PST - ppublish
SO  - N Engl J Med. 1989 Jul 27;321(4):213-8. doi: 10.1056/NEJM198907273210402.