PMID- 1983211
OWN - NLM
STAT- MEDLINE
DCOM- 19910918
LR  - 20101118
IS  - 0261-2429 (Print)
IS  - 0261-2429 (Linking)
VI  - 9
IP  - 4
DP  - 1990
TI  - Multiple endocrine neoplasia.
PG  - 703-23
AB  - The recent chromosomal mapping of the genes for two different autosomal dominant 
      inherited predispositions to multiple endocrine neoplasias promises to be a 
      significant breakthrough for the understanding of the pathogenesis of such 
      lesions. Multiple endocrine neoplasia type 1 (MEN1) associates primary 
      hyperparathyroidism, lesions of the endocrine pancreas and pituitary adenomas. 
      The first hint that the MEN1 gene is localized on chromosome 11 came from the 
      finding of allele losses in MEN1 associated tumours. Subsequent genetic linkage 
      analysis to restriction fragment length polymorphism (RFLP) markers assigned the 
      gene to chromosome band 11q13. MEN2A is characterized by medullary thyroid 
      carcinoma and phaeochromocytoma. The disease locus was localized to the 
      centromeric region of chromosome 10 by genetic linkage. For both syndromes 
      genetic linkage maps of the flanking regions have been established, and a set of 
      RFLP markers is now available for premorbid identification of gene carriers in 
      affected families. Analysis of allele losses showed that tumorigenesis of 
      parathyroid and pancreatic lesions results from unmasking of a recessive mutation 
      at the MEN1 locus, and by deletion mapping the tentative MEN1 region was 
      restricted to a few million base pairs. In contrast, such losses appear to be 
      relatively rare in MEN2A associated lesions.
FAU - Larsson, C
AU  - Larsson C
AD  - Department of Clinical Genetics, Karolinska Hospital, Stockholm, Sweden.
FAU - Nordenskjold, M
AU  - Nordenskjold M
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Review
PL  - United States
TA  - Cancer Surv
JT  - Cancer surveys
JID - 8218015
SB  - IM
MH  - Adrenal Gland Neoplasms/genetics
MH  - *Chromosome Mapping
MH  - Genetic Linkage
MH  - Heterozygote
MH  - Humans
MH  - Multiple Endocrine Neoplasia/*genetics
MH  - Pituitary Neoplasms/genetics
RF  - 74
EDAT- 1990/01/01 00:00
MHDA- 1990/01/01 00:01
CRDT- 1990/01/01 00:00
PHST- 1990/01/01 00:00 [pubmed]
PHST- 1990/01/01 00:01 [medline]
PHST- 1990/01/01 00:00 [entrez]
PST - ppublish
SO  - Cancer Surv. 1990;9(4):703-23.