Role of chromatin remodeling gene Cecr2 in neurulation and inner ear development.
The loss of Cecr2, which encodes a chromatin remodeling protein, has been associated with the neural tube defect (NTD) exencephaly and open eyelids in mice. Here, we show that two independent mutations of Cecr2 are also associated with specific inner ear defects. Homozygous mutant 18.5 days post coitus (dpc) fetuses exhibited smaller cochleae as well as rotational defects of sensory cells and extra cell rows in the inner ear reminiscent of planar cell polarity (PCP) mutants. Cecr2 was expressed in the neuroepithelium, head mesenchyme, and the cochlear floor. Although limited genetic interaction for NTDs was seen with Vangl2, a microarray analysis of PCP genes did not reveal a direct connection to this pathway. The mechanism of Cecr2 action in neurogenesis and inner ear development is likely complex.
Department of Biological Sciences, University of Alberta, Edmonton, Alberta, Canada. cedawe@ualberta.ca
Christine E
CE
Dawe
Megan K
MK
Kooistra
Nicholas A
NA
Fairbridge
Amanda C
AC
Pisio
Heather E
HE
McDermid
D000818:Animals; D016764:Cell Polarity / Q000235:genetics; D002843:Chromatin / Q000378:metabolism*; D007758:Ear, Inner / Q000033:anatomy & histology* / Q000196:embryology* / Q000378:metabolism / Q000502:physiology; D004622:Embryo, Mammalian / Q000033:anatomy & histology / Q000502:physiology; D005260:Female; D006801:Humans; D036341:Intercellular Signaling Peptides and Proteins / Q000235:genetics* / Q000378:metabolism; D008297:Male; D008648:Mesoderm / Q000378:metabolism; D051379:Mice; D008807:Mice, Inbred BALB C; D008810:Mice, Inbred C57BL; D046228:Microarray Analysis; D009154:Mutation; D009419:Nerve Tissue Proteins / Q000235:genetics; D009436:Neural Tube Defects / Q000235:genetics; D046569:Neuroepithelial Cells / Q000378:metabolism; D054261:Neurulation / Q000502:physiology*; D038081:Organogenesis / Q000235:genetics*; D014157:Transcription Factors
C501837:CECR2 protein, mouse; D002843:Chromatin; D036341:Intercellular Signaling Peptides and Proteins; C431577:Ltap protein, mouse; D009419:Nerve Tissue Proteins; D014157:Transcription Factors