PMID- 11158604
OWN - NLM
STAT- MEDLINE
DCOM- 20010412
LR  - 20220419
IS  - 0027-8424 (Print)
IS  - 1091-6490 (Electronic)
IS  - 0027-8424 (Linking)
VI  - 98
IP  - 3
DP  - 2001 Jan 30
TI  - A mouse model of multiple endocrine neoplasia, type 1, develops multiple 
      endocrine tumors.
PG  - 1118-23
AB  - Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant cancer 
      syndrome, characterized primarily by multiple tumors in the parathyroid glands, 
      endocrine pancreas, and anterior pituitary. Other tumors, including gastrinoma, 
      carcinoid, adrenal cortical tumors, angiofibroma, collagenoma, and lipoma, also 
      occur in some patients. Individuals with MEN1 almost always have loss-of-function 
      mutations in the MEN1 gene on chromosome 11, and endocrine tumors arising in 
      these patients usually show somatic loss of the remaining wild-type allele. To 
      examine the role of MEN1 in tumor formation, a mouse model was generated through 
      homologous recombination of the mouse homolog Men1. Homozygous mice die in utero 
      at embryonic days 11.5-12.5, whereas heterozygous mice develop features 
      remarkably similar to those of the human disorder. As early as 9 months, 
      pancreatic islets show a range of lesions from hyperplasia to insulin-producing 
      islet cell tumors, and parathyroid adenomas are also frequently observed. Larger, 
      more numerous tumors involving pancreatic islets, parathyroids, thyroid, adrenal 
      cortex, and pituitary are seen by 16 months. All of the tumors tested to date 
      show loss of the wild-type Men1 allele, further supporting its role as a tumor 
      suppressor gene.
FAU - Crabtree, J S
AU  - Crabtree JS
AD  - National Human Genome Research Institute, National Cancer Institute, and National 
      Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of 
      Health, Bethesda, MD 20892, USA.
FAU - Scacheri, P C
AU  - Scacheri PC
FAU - Ward, J M
AU  - Ward JM
FAU - Garrett-Beal, L
AU  - Garrett-Beal L
FAU - Emmert-Buck, M R
AU  - Emmert-Buck MR
FAU - Edgemon, K A
AU  - Edgemon KA
FAU - Lorang, D
AU  - Lorang D
FAU - Libutti, S K
AU  - Libutti SK
FAU - Chandrasekharappa, S C
AU  - Chandrasekharappa SC
FAU - Marx, S J
AU  - Marx SJ
FAU - Spiegel, A M
AU  - Spiegel AM
FAU - Collins, F S
AU  - Collins FS
LA  - eng
PT  - Journal Article
PL  - United States
TA  - Proc Natl Acad Sci U S A
JT  - Proceedings of the National Academy of Sciences of the United States of America
JID - 7505876
RN  - 0 (MEN1 protein, human)
RN  - 0 (Neoplasm Proteins)
RN  - 0 (Proto-Oncogene Proteins)
RN  - EC 2.7.2.3 (Phosphoglycerate Kinase)
SB  - IM
MH  - Adenoma/genetics/pathology
MH  - Animals
MH  - Chromosome Mapping
MH  - Chromosomes, Artificial, Bacterial
MH  - Chromosomes, Human, Pair 11
MH  - Crosses, Genetic
MH  - Disease Models, Animal
MH  - Exons
MH  - Female
MH  - Fetal Death
MH  - Genes, Lethal
MH  - Genes, Tumor Suppressor
MH  - Homozygote
MH  - Humans
MH  - Hyperparathyroidism/genetics/pathology
MH  - Islets of Langerhans/pathology
MH  - Male
MH  - Mice
MH  - Mice, Inbred Strains
MH  - Mice, Transgenic
MH  - Multiple Endocrine Neoplasia Type 1/*genetics/pathology
MH  - Neoplasm Proteins/*genetics
MH  - Pancreatic Neoplasms/genetics/pathology
MH  - Parathyroid Neoplasms/genetics/pathology
MH  - Phosphoglycerate Kinase/genetics
MH  - Pregnancy
MH  - *Proto-Oncogene Proteins
MH  - Recombination, Genetic
PMC - PMC14718
EDAT- 2001/02/07 11:00
MHDA- 2001/04/17 10:01
PMCR- 2001/07/30
CRDT- 2001/02/07 11:00
PHST- 2001/02/07 11:00 [pubmed]
PHST- 2001/04/17 10:01 [medline]
PHST- 2001/02/07 11:00 [entrez]
PHST- 2001/07/30 00:00 [pmc-release]
AID - 98/3/1118 [pii]
AID - 5655 [pii]
AID - 10.1073/pnas.98.3.1118 [doi]
PST - ppublish
SO  - Proc Natl Acad Sci U S A. 2001 Jan 30;98(3):1118-23. doi: 10.1073/pnas.98.3.1118.