Functional studies of the MEN1 gene

被引:51
作者
Chandrasekharappa, SC [1 ]
Teh, BT
机构
[1] NHGRI, Genome Technol Branch, NIH, Bethesda, MD 20892 USA
[2] Van Andel Res Inst, Canc Genet Lab, Grand Rapids, MI USA
关键词
Jun D; menin; multiple endocrine neoplasia type 1 (MEN1); nuclear localization; protein interaction; tumour suppressor;
D O I
10.1046/j.1365-2796.2003.01165.x
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Multiple endocrine neoplasia type 1 is an autosomal dominant cancer syndrome affecting primarily parathyroid, enteropancreatic endocrine and pituitary tissues. The inactivating germline and somatic mutations spread throughout the gene and the accompanying loss of the second allele in tumours show that the MEN1 gene is a tumour suppressor. The MEN1 -encoded protein, menin, is a novel nuclear protein. Menin binds and alters JunD-, NF-kappaB-, Smad3-mediated transcriptional activation. The mouse Men1 knockout model mimicks the human MEN1 condition contributing to the understanding of tumorigenesis in MEN1.
引用
收藏
页码:606 / 615
页数:10
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