Piccolino is required for ribbon architecture at cochlear inner hair cell synapses and for hearing.

Autor: Michanski S; Molecular Architecture of Synapses Group, Institute for Auditory Neuroscience and InnerEarLab, University Medical Center Göttingen, Göttingen, Germany.; Center for Biostructural Imaging of Neurodegeneration, University Medical Center Göttingen, Göttingen, Germany.; Collaborative Research Center 889 'Cellular Mechanisms of Sensory Processing', Göttingen, Germany.; Multiscale Bioimaging of Excitable Cells, Cluster of Excellence, Göttingen, Germany., Kapoor R; Institute for Auditory Neuroscience and InnerEarLab, University Medical Center Göttingen, Göttingen, Germany.; Auditory Neuroscience and Synaptic Nanophysiology Group, Max Planck Institute for Multidisciplinary Sciences, Göttingen, Germany.; IMPRS Molecular Biology, Göttingen Graduate School for Neuroscience and Molecular Biosciences, University of Göttingen, Göttingen, Germany., Steyer AM; Electron Microscopy Core Unit, Department of Neurogenetics, Max Planck Institute for Multidisciplinary Sciences, Göttingen, Germany., Möbius W; Multiscale Bioimaging of Excitable Cells, Cluster of Excellence, Göttingen, Germany.; Electron Microscopy Core Unit, Department of Neurogenetics, Max Planck Institute for Multidisciplinary Sciences, Göttingen, Germany., Früholz I; Developmental, Neural, and Behavioral Biology Master Program, University of Göttingen, Göttingen, Germany., Ackermann F; German Center for Neurodegenerative Diseases, Berlin, Germany., Gültas M; Faculty of Agriculture, South Westphalia University of Applied Sciences, Soest, Germany., Garner CC; German Center for Neurodegenerative Diseases, Berlin, Germany.; NeuroCureCluster of Excellence, Charité - Universitätsmedizin, Berlin, Germany., Hamra FK; Department of Obstetrics and Gynecology, University of Texas Southwestern Medical Center, Dallas, TX, USA., Neef J; Collaborative Research Center 889 'Cellular Mechanisms of Sensory Processing', Göttingen, Germany.; Institute for Auditory Neuroscience and InnerEarLab, University Medical Center Göttingen, Göttingen, Germany.; Auditory Neuroscience and Synaptic Nanophysiology Group, Max Planck Institute for Multidisciplinary Sciences, Göttingen, Germany., Strenzke N; Collaborative Research Center 889 'Cellular Mechanisms of Sensory Processing', Göttingen, Germany.; Auditory Systems Physiology Group, Institute for Auditory Neuroscience and InnerEarLab, University Medical Center Göttingen, Göttingen, Germany., Moser T; Collaborative Research Center 889 'Cellular Mechanisms of Sensory Processing', Göttingen, Germany.; Multiscale Bioimaging of Excitable Cells, Cluster of Excellence, Göttingen, Germany.; Institute for Auditory Neuroscience and InnerEarLab, University Medical Center Göttingen, Göttingen, Germany.; Auditory Neuroscience and Synaptic Nanophysiology Group, Max Planck Institute for Multidisciplinary Sciences, Göttingen, Germany., Wichmann C; Molecular Architecture of Synapses Group, Institute for Auditory Neuroscience and InnerEarLab, University Medical Center Göttingen, Göttingen, Germany.; Center for Biostructural Imaging of Neurodegeneration, University Medical Center Göttingen, Göttingen, Germany.; Collaborative Research Center 889 'Cellular Mechanisms of Sensory Processing', Göttingen, Germany.; Multiscale Bioimaging of Excitable Cells, Cluster of Excellence, Göttingen, Germany.
Jazyk: angličtina
Zdroj: EMBO reports [EMBO Rep] 2023 Sep 06; Vol. 24 (9), pp. e56702. Date of Electronic Publication: 2023 Jul 21.
DOI: 10.15252/embr.202256702
Abstrakt: Cochlear inner hair cells (IHCs) form specialized ribbon synapses with spiral ganglion neurons that tirelessly transmit sound information at high rates over long time periods with extreme temporal precision. This functional specialization is essential for sound encoding and is attributed to a distinct molecular machinery with unique players or splice variants compared to conventional neuronal synapses. Among these is the active zone (AZ) scaffold protein piccolo/aczonin, which is represented by its short splice variant piccolino at cochlear and retinal ribbon synapses. While the function of piccolo at synapses of the central nervous system has been intensively investigated, the role of piccolino at IHC synapses remains unclear. In this study, we characterize the structure and function of IHC synapses in piccolo gene-trap mutant rats (Pclo gt/gt ). We find a mild hearing deficit with elevated thresholds and reduced amplitudes of auditory brainstem responses. Ca 2+ channel distribution and ribbon morphology are altered in apical IHCs, while their presynaptic function seems to be unchanged. We conclude that piccolino contributes to the AZ organization in IHCs and is essential for normal hearing.
(© 2023 The Authors.)
Databáze: MEDLINE
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