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Background information . The BOR (branchio‐oto‐renal) syndrome is a dominant disorder most commonly caused by mutations in the  EYA1  (Eyes Absent 1) gene. Symptoms commonly include deafness and renal anomalies.   Results . We have used the embryos of the frog  Xenopus laevis  as an animal model for early ear development to examine the effects of different  EYA1  mutations. Four  eya1  mRNAs encoding proteins correlated with congenital anomalies in human were injected into early stage embryos. We show that the expression of mutations associated with BOR, even in the presence of normal levels of endogenous  eya1  mRNA, leads to morphologically abnormal ear development as measured by overall otic vesicle size, establishment of sensory tissue and otic innervation. The molecular consequences of mutant eya1 expression were assessed by QPCR (quantitative PCR) analysis and  in situ  hybridization. Embryos expressing mutant eya1 showed altered levels of multiple genes ( six1 ,  dach ,  neuroD ,  ngnr‐1  and  nt3 ) important for normal ear development.   Conclusions . These studies lend support to the hypothesis that dominant‐negative effects of  EYA1  mutations may have a role in the pathogenesis of BOR.

EYA1 mutations associated with the branchio‐oto‐renal syndrome result in defective otic development in Xenopus laevis