Phenotypes for Chd7 Tg(Atoh1-cre)1Bfri MGI:7339183 MGI Mouse

increased neuron apoptosis ( J:242933 )

• increased granule cell precursor apoptosis in both the cerebellar vermis and hemispheres at P7, but is only statistically significant in the hemispheres

decreased cerebellar granule cell precursor proliferation ( J:242933 )

• in vermis lobules I - VIII at early postnatal stages, but not in the hemispheres

cochlear inner hair cell degeneration ( J:314588 )

• rapid degeneration of inner hair cells at P10 and P14 in 6 of 8 and 15 of 16 mice, respectively

• however, inner hair cell development and morphology are similar to controls at P7

• by P21 most nuclei are missing, pyknotic, or fragmented

cochlear outer hair cell degeneration ( J:314588 )

• degeneration of outer hair cells is slower than that of inner hair cells

abnormal cerebellar foliation ( J:242933 )

• irregular

• delayed initiation of foliation in the vermis becomes evident at E17.5

abnormal cerebellar hemisphere morphology ( J:242933 )

• hypoplasia in the hemispheres evident by P7

abnormal Purkinje cell morphology ( J:242933 )

• at P7 and P21 cells in the cerebellar vermis are organized in monolayers with small regions of slightly disorganized cells

• at P7, large patches of Purkinje cells are present in the cerebellar hemispheres

• distribution is irregular at E16.5 and mislocalized cells are evident in the cerebellar vermis and hemispheres by E18.5

decreased Purkinje cell number ( J:242933 )

• in the cerebella at P21

• decrease is due to reduction in the number of cells in lobules I-VII

abnormal cerebellum vermis lobule morphology ( J:242933 )

• vermis folia IV-V and IX extend abnormally into the hemispheres

cerebellum vermis hypoplasia ( J:242933 )

• becomes evident at E18.5

cerebellum hypoplasia ( J:242933 )

• at P21, with hypoplasia in lobules I - VIII, most strikingly in the central lobules

• becomes evident in the vermis at E18.5 and in the hemispheres by P7

behavior/neurological phenotype ( J:242933 )

N	• no differences in vocalization, social investigation or olfactory discrimination

impaired righting response ( J:242933 )

• delay in acquiring the righting reflex

impaired coordination ( J:242933 )

• males but not females perform worse in a rotarod assay compared to sex-matched control littermates

behavioral developmental delay ( J:242933 )

• delay in acquiring the righting reflex. negative geotaxis, ability to reach out toward an object

increased cellular sensitivity to oxidative stress ( J:314588 )

• when explants of P6 cochlea are exposed to gentamicin for 5h more than half of the hair cells are lost, similar treatment of control cultures does not result in hair cell loss