Six novel MEN1 gene mutations in sporadic parathyroid tumors

Hum Mutat. 2000 Nov;16(5):445. doi: 10.1002/1098-1004(200011)16:5<445::AID-HUMU12>3.0.CO;2-6.

Abstract

We report nine mutations of the multiple endocrine neoplasia type 1 (MEN1) gene in sporadic parathyroid adenomas. Six of them have not previously been described: E60X, P32R, 261delA, 934+2T-->G, S443P, and 1593insC. The tissue samples were initially submitted to LOH analysis at 11q13 followed by SSCP screening of LOH-positive samples. Mutations were identified by direct sequencing and subcloning. Three (E60X, P32R, and 261delA) were in exon 2, one (934+2bp) in the splice junction of exon 5, one (S443P) in exon 9, and one (1593insC) in exon 10. The 3 mutations in exon 2 were associated with loss and/or creation of a restriction site. The corresponding germline sequence of the MEN1 gene was normal. Most mutations would likely result in a nonfunctional menin protein, and therefore in the loss of a tumor suppressor protein.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adenoma / genetics
  • Aged
  • Genes, Tumor Suppressor / genetics
  • Humans
  • Male
  • Multiple Endocrine Neoplasia Type 1 / genetics*
  • Mutagenesis, Insertional / genetics
  • Mutation / genetics*
  • Mutation, Missense / genetics
  • Neoplasm Proteins / genetics
  • Parathyroid Neoplasms / genetics*
  • Proto-Oncogene Proteins*
  • Sequence Deletion / genetics

Substances

  • MEN1 protein, human
  • Neoplasm Proteins
  • Proto-Oncogene Proteins