Gfi11b/1b-mouse model – A model for studying hearing loss

Gfi1 is a transcriptional repressor being expressed in the hematopoietic and nervous system. Loss of function leads to severe defects in hematopoiesis and inner ear development.

Gfi1 is highly similar to its paralogue Gfi1b with almost identical sequences in their respective protein domains. Technology A knock-in mouse model was created in which the Gfi1 coding region was replaced by Gfi1b. The Gfi11b/1b-mice did not exhibit significant defects in the hematopoietic system as previously described for Gfi1-/–mice, suggesting that Gfi1b is able to rescue the loss of Gfi1-function. Interestingly, however, the Gfi11b/1b-mice showed significant defects in inner ear development, i.e. hair cell formation, leading to hearing loss. These results point to some interesting mechanisms on the molecular level for the differentiation and maintenance of hearing. This makes the Gfi11b/1b-model a valuable tool for the development of novel approaches for treating hearing loss.

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