Human Molecular Genetics, 2000, Vol. 9, No. 3 413-419
© 2000 Oxford University Press
Mice deficient in the candidate tumor suppressor gene Hic1 exhibit developmental defects of structures affected in the Miller–Dieker syndrome
1Graduate Program in Human Genetics and Molecular Biology, 2Graduate Program in Cellular and Molecular Medicine, 3Division of Comparative Medicine, 4Department of Pathology and 5Department of Molecular Microbiology and Immunology, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA, 6Transgenic and Knockout Facility, National Institute on Aging, National Institutes of Health, Baltimore, MD 21224-6825, USA, 7The Oncology Center, The Johns Hopkins Medical Institutions, 424 North Bond Street, Baltimore, MD 21231, USA
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.
+ To whom correspondence should be addressed. Tel: +1 410 955 8506; Fax: +1 410 614 9884; Email: sbaylin@welchlink.welch.jhu.edu
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