A KRAB domain zinc finger protein in imprinting and disease.
The monoallelic expression of imprinted genes is regulated by DNA methylation marks that originate from the oocyte or sperm. Li et al. (2008) show in this issue of Developmental Cell that the KRAB zinc finger protein Zfp57 contributes to the embryonic maintenance of these imprints. At one locus, Zfp57 is also involved in imprint establishment. These findings provide a mechanistic interpretation for Mackay et al.'s recently reported ZFP57 mutations in patients with transient neonatal diabetes.
Institute of Molecular Genetics, CNRS and University of Montpellier, 1919 Route de Mende, 34293 Montpellier, France.
Ryutaro
R
Hirasawa
Robert
R
Feil
D000818:Animals; D003920:Diabetes Mellitus / Q000235:genetics*; D018392:Genomic Imprinting; D006801:Humans; D007231:Infant, Newborn; D051379:Mice; D018345:Mice, Knockout; D009154:Mutation; D017434:Protein Structure, Tertiary; D012097:Repressor Proteins / Q000235:genetics* / Q000378:metabolism
D012097:Repressor Proteins; C501529:Zfp-57 protein, mouse