• Otoferlin plays a critical roles in exocytosis and vesicle replenishment in inner hair cells of cochlea. If the function of Otoferlin protein is impaired, the IHC will be unable to transmit the electrical signals to the auditory ganglion neurons, leading to profound deafness.
• The genome sequences between exons 3-46 of mouse Otoferlin gene were depleted in B-Otoferlin KO mice, resulting in a disruption of the mouse Otoferlin gene.
• Click and pure-tone ABR showed the homozygous B-Otoferlin KO mice are profound hearing loss.
• This product is used for gene therapy of hearing disorder caused by disruption of Otoferlin gene.

Gene targeting strategy for B-Otoferlin KO mice. The genome sequences between exons 3-46 of mouse Otoferlin gene were depleted in B-Otoferlin KO mice, resulting in a disruption of the mouse Otoferlin gene.

Auditory brainstem response thresholds for wild-type C57BL/6JNifdc and B-Otoferlin KO mice. Both ears from wild-type C57BL/6JNifdc and homozygous B-Otoferlin KO mice (10-week-old, 2 male and 2 female) were evaluated the auditory brainstem responses. The click stimuli ABR (A) and pure-tone stimuli of 4kHz, 8kHz, 12kHz, 16kHz, 24kHz and 32kHz ABR (B) thresholds of B-Otoferlin KO mice showed the homozygous B-Otoferlin KO mice are profound hearing loss. Values are expressed as mean ± SEM.

Those data were completed with our cooperation partners.