Premature Centromere Division in Patients with Multiple Endocrine Neoplasia Type 1

Frequency of mitoses with premature centromere division (PCD) was examined in lymphocytes from subjects with multiple endocrine neoplasia type 1 (MEN 1). An increase in PCD after exposure to an alkylating agent was observed in subjects with MEN 1 who carry a heterozygous MEN1 gene mutation but not in normal controls or in affected subjects without the MEN1 gene mutation. These findings support the inclusion of MEN 1 as a chromosome instability syndrome and recognition of PCD as a manifestation of chromosome instability. Furthermore, these results suggest that the MEN1 gene product may function to maintain the integrity of DNA.