What are significant findings on DFNB9 mice models?
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DFNB9 mice models have shown significant findings related to the restoration of otoferlin expression and the reversal of the deafness phenotype through gene therapy using adeno-associated virus (AAV) vectors . Another study on DFNB9 mice models revealed that the lack of calcium-binding proteins (Cabp1 and Cabp2) led to impaired synaptic transmission and elevated auditory brainstem response (ABR) thresholds, but AAV-mediated Cabp2 expression partially restored calcium channel function and improved ABR thresholds . Additionally, the study found that reduced signaling of brain-derived neurotrophic factor (BDNF) receptor TrkB aggravated memory impairment in DFNB9 mice models, and BDNF protein levels were reduced in the cortex of these mice . Furthermore, DFNB91 mice models lacking the Serpinb6a gene exhibited progressive hearing loss, which was delayed when transferred onto a Cdh23 normal background, suggesting that Serpinb6 helps maintain hearing by protecting hair cells from stress . Lastly, Coch(G88E/G88E) mice models for DFNA9 showed elevated vestibular-evoked potential (VsEP) thresholds and progressive hearing loss, providing an opportunity to study age-related hearing loss and potential therapies .
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| Papers (5) | Insight |
|---|---|
| The provided paper is about a Coch(G88E/G88E) mouse model for DFNA9 late-onset hearing loss and vestibular dysfunction. The significant findings on the DFNA9 mice models include elevated vestibular-evoked potential (VsEP) thresholds, substantially elevated auditory brainstem response (ABR) thresholds at 21 months, and lower distortion product otoacoustic emission (DPOAE) amplitudes. | |
3 Citations | The paper discusses the use of a mouse model to study DFNB91, a form of progressive hearing loss in humans. The study shows that mice lacking the Serpinb6a gene exhibit early-onset hearing loss, which can be delayed by transferring the mutant allele onto a different mouse strain. The mice also show increased susceptibility to permanent hearing loss after exposure to acoustic trauma. |
52 Citations | The provided paper is about the impact of Bdnf gene deficiency on memory impairment and brain pathology in a mouse model of Alzheimer's disease. There is no mention of DFNB9 mice models in the paper. |
| The significant findings on DFNB9 mice models include the restoration of otoferlin expression and the reversal of the deafness phenotype through a dual AAV-mediated gene therapy approach. | |
| The provided paper is about a mouse model for DFNB93, not DFNB9. Therefore, there is no information about significant findings on DFNB9 mice models in the paper. |
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