Deciphering Auditory Hyperexcitability in Otogl Mutant Mice Unravels an Auditory Neuropathy Mechanism.

• Tác giả: Paul Avan, Brice Bathellier, Carolina Campos Pina, Sedigheh Delmaghani, Typhaine Dupont, Aziz El-Amraoui, Raphaël Etournay, Mathilde Gagliardini, Fabrice Giraudet, Boris Gourévitch, Talya C Inbar, Philippe Jean, Baptiste Libé-Philippot, Adeline Mallet, Sabrina Mechaussier, Nicolas Michalski, Monica Morais, Christine Petit, Olivier Postal, Cyril Scandola, Joanna Schwenkgrub, Amrit Singh-Estivalet, Carla Maria Barbosa Spinola, Shéhanie Udugampolage, Elisabeth Verpy
• Ngôn ngữ: eng
• Ký hiệu phân loại: 940.531709 1918
• Thông tin xuất bản: Germany : Advanced science (Weinheim, Baden-Wurttemberg, Germany) , 2025
• Bộ sưu tập: NCBI
• ID: 199002

Tóm tắt

Auditory neuropathies affect the spiral ganglion neurons of the auditory nerve or their synapses with the sensory hair cells, distorting the sound information transmitted from the ear to the brain. Deciphering the underlying pathophysiological mechanisms remains challenging owing to the diversity of spiral ganglion neuron subtypes and associated central auditory circuits. An auditory neuropathy mechanism is unraveled by investigating the origin of auditory hyperexcitability in a mouse model for hereditary congenital deafness. Otogl encodes the large Otogelin-like protein, which is related to secreted epithelial mucins and is implicated in the mechanical stimulation of cochlear outer hair cells. Heterozygous Otogl