Mouse Genome Informatics
hm
    Six1Cwe/Six1Cwe
C3HeB/FeJ-Six1Cwe
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
hearing/vestibular/ear
• at E16.5
• the cochlea is severely truncated and oval and round windows are absent compared to in wild-type mice
• at E16.5, development of hair cells is disrupted compared to in wild-type mice
• at P21, utricles exhibit few hair cells unlike in wild-type mice
• at P21, mice lack sensory hair cells in the cristae ampulari and rudimentary cochlea unlike in wild-type mice
• however, the utricle contained a small patch of scattered hair cells
• ear canals are thin or truncated compared to in wild-type mice
• the anterior canal is truncated at the ampullar end unlike in wild-type mice
• the posterior crista ampularis is absent

behavior/neurological
• when held by their tail, mice curl up towards their belly and displayed jiggling behavior unlike wild-type mice
• extreme

growth/size

renal/urinary system
• 41% of mice exhibit a unilateral hypoplastic kidney

craniofacial
• at E16.5

skeleton
• at E16.5

nervous system
• at E16.5, development of hair cells is disrupted compared to in wild-type mice
• at P21, utricles exhibit few hair cells unlike in wild-type mice
• at P21, mice lack sensory hair cells in the cristae ampulari and rudimentary cochlea unlike in wild-type mice
• however, the utricle contained a small patch of scattered hair cells

Mouse Models of Human Disease
OMIM IDRef(s)
Branchiootorenal Syndrome 1; BOR1 113650 J:149467