HIC1 is a candidate tumor suppressor gene which is frequently hypermethylated in 
human tumors, and its location within the Miller-Dieker syndrome's critical 
deletion region at chromosome 17p13.3 makes it a candidate gene for involvement 
in this gene deletion syndrome. To study the function of murine Hic1 in 
development, we have created Hic1 -deficient mice. These animals die perinatally 
and exhibit varying combinations of gross developmental defects throughout the 
second half of development, including acrania, exencephaly, cleft palate, limb 
abnormalities and omphalocele. These findings demonstrate a role for Hic1 in the 
development of structures affected in the Miller-Dieker syndrome, and provide 
functional evidence to strengthen its candidacy as a gene involved in this 
disorder.