Multiple endocrine neoplasia type 1 (MEN1) in two Asian families

✍ Scribed by B. T. Teh; S. I. Hii; R. David; V. Parameswaran; S. Grimmond; M. K. Walters; T. T. Tan; D. J. Nancarrow; S. P. Chan; J. Mennon; C. Larsson; A. Zaini; B. A. K. Khalid; J. J. Shepherd; D. P. Cameron; N. K. Hayward

• Publisher: Springer
• Year: 1994
• Tongue: English
• Weight: 453 KB
• Volume: 94
• Category: Article
• ISSN: 0340-6717
• DOI: 10.1007/bf00211009

✦ Synopsis

Multiple endocrine neoplasia type 1 (MEN1), an autosomal dominant disease characterized by neoplasia of the parathyroid glands, anterior pituitary and endocrine pancreas, is rarely reported in Asian populations. The MEN1 gene, mapped to chromosome 11q13 but yet to be cloned, has been found to be homogeneous in Caucasian populations through linkage analysis. Here, two previously unreported Asian kindreds with MEN1 are described; linkage analysis using microsatellite polymorphic markers in the MEN1 region was carried out. The first kindred, of Mongolian-Chinese origin, is a multigeneration family with over 150 living members, eight of whom are affected to date. The second kindred is of Chinese origin consisting of four affected members. Linkage to chromosome 11q13 was confirmed in both kindreds, supporting evidence for genetic homogeneity. A recombination in the larger kindred localizes the gene distal to marker D11S956, consistent with its placement from previous studies. We also show that it is feasible to use these markers for predictive testing, as four gene carriers were detected in 13 family members with unknown disease status in the first kindred.