First published online February 24, 2006
Development 133, 605e (2006)
© The Company of Biologists Limited
Dnmt3b in development and ICF
Widespread de novo DNA methylation normally occurs during embryonic
development but which genes are regulated by this epigenetic process is
unknown. Now clues to their identity may come from the two mouse strains
described on p. 1183
by En Li and colleagues. These mice carry missense mutations in DNA
methyltransferase 3B (Dnmt3b). In humans, DNMT3B mutations
cause a rare inherited disorder called ICF (for immunodeficiency, centromeric
instability and facial anomalies) syndrome. To create an animal model of ICF
and to investigate the role of Dnmt3b in mouse development, the researchers
generated mice with a null Dnmt3b allele and also mice carrying one
of two ICF-like missense mutations. The Dnmt3b null allele caused
embryonic lethality, but mice carrying homozygous ICF mutations survived to
term and exhibited a phenotype that resembled that of human ICF patients. The
researchers conclude that Dnmt3b is essential for mouse embryonic development
and propose that ICF missense mutations cause only a partial loss of DNMT3B
function.
Related articles in Development:
- Roles for Dnmt3b in mammalian development: a mouse model for the ICF syndrome
- Yoshihide Ueda, Masaki Okano, Christine Williams, Taiping Chen, Katia Georgopoulos, and En Li
Development 2006 133: 1183-1192.
[Abstract]
[Full Text]
