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. 2009 Sep 18;5(9):e1000650. doi: 10.1371/journal.pgen.1000650

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This is an open-access article distributed under the terms of the Creative Commons Public Domain declaration which stipulates that, once placed in the public domain, this work may be freely reproduced, distributed, transmitted, modified, built upon, or otherwise used by anyone for any lawful purpose.

This is an open-access article distributed under the terms of the Creative Commons Public Domain declaration, which stipulates that, once placed in the public domain, this work may be freely reproduced, distributed, transmitted, modified, built upon, or otherwise used by anyone for any lawful purpose.

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(A) Measurements from micro-CT analysis (Figure 3) reveal a 25% reduction in endocranial volume in Nipbl+/− mice (data are means±SD from 40 wildtype and 23 mutant skulls). (B) Nissl-stained coronal sections of adult wildtype (+/+) and Nipbl+/− brains illustrate reduced brain size, but grossly normal neuroanatomy. MHb, medial habenular nucleus; fr, fasiculus retroflexus; mt, mammillothalamic tract; dg, dentate gyrus; cc, corpus callosum. Scale bar: 1 mm. (C) Cerebellar hypoplasia in Nipbl+/− mice. Cresyl violet-stained midsagittal sections through the cerebellum of adult wildtype and Nipbl+/− mice. Mutant cerebella are smaller overall, with a less well-developed folium IX (bracket); note the subfolium (arrow in +/+) that was missing in 100% of analyzed mutants (asterisk; N = 3 Nipbl+/− and 3 wildtype littermate controls assessed). Reduction in the size of folium I was also commonly observed in mutants (not shown). Scale bar: 500 µm. (D,E) Corneal pathology in Nipbl+/− mice. (D) External view of wildtype and Nipbl+/− eyes, demonstrating central opacity (green arrowhead) and swelling/inflammation in the periorbital area (arrows) in the mutant. (E) Sections through wildtype and Nipbl+/− eyes, demonstrating disruption of corneal structure in the mutant, including infiltration of cells into the stroma and loss of epithelium. Ocular opacification was observed in 14% (24/173) of post-weaning animals tested. Scale bar: 100 µm. (F,G) Hearing deficits in Nipbl+/− mice. (F) Auditory brainstem evoked response (ABR) records for a pair of wildtype and Nipbl+/− littermates, performed as described [62]. Stacked curves are responses to successive 10 dB increments of a pure-tone stimulus, and display five characteristic peaks of differing latency. In the Nipbl+/− curves, hearing loss is indicated by the much higher response threshold (this is seen in less than half of mutants). (G) Average background-subtracted sizes of Peaks II, III, and IV (normalized to Peak I to correct for experimental variation due to differences in electrode placement) for the 90 dB tone response of 6 wildtype and 6 mutant animals. Mutants show marked depression of peak III (P<0.02, ANOVA), consistent with abnormalities at the level of the auditory nerve or brainstem.