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@ARTICLE{Michanski:263802,
author = {Michanski, Susann and Kapoor, Rohan and Steyer, Anna M and
Möbius, Wiebke and Früholz, Iris and Ackermann, Frauke and
Gültas, Mehmet and Garner, Craig C and Hamra, F Kent and
Neef, Jakob and Strenzke, Nicola and Moser, Tobias and
Wichmann, Carolin},
title = {{P}iccolino is required for ribbon architecture at cochlear
inner hair cell synapses and for hearing.},
journal = {EMBO reports},
volume = {24},
number = {9},
issn = {1469-221X},
address = {Hoboken, NJ [u.a.]},
publisher = {Wiley},
reportid = {DZNE-2023-00888},
pages = {e56702},
year = {2023},
abstract = {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 (Pclogt/gt ).
We find a mild hearing deficit with elevated thresholds and
reduced amplitudes of auditory brainstem responses. Ca2+
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.},
keywords = {Rats / Animals / Hair Cells, Auditory, Inner / Hearing:
physiology / Synapses: physiology / Cochlea / Spiral
Ganglion: metabolism / Cytoskeletal Proteins: metabolism /
Neuropeptides / Ca2+ channel (Other) / active zone (Other) /
cochlea (Other) / hearing (Other) / synaptic vesicle (Other)
/ Pclo protein, rat (NLM Chemicals) / Cytoskeletal Proteins
(NLM Chemicals) / Neuropeptides (NLM Chemicals)},
cin = {AG Ackermann / AG Garner},
ddc = {570},
cid = {I:(DE-2719)1813004 / I:(DE-2719)1810001},
pnm = {351 - Brain Function (POF4-351)},
pid = {G:(DE-HGF)POF4-351},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:37477166},
pmc = {pmc:PMC10481675},
doi = {10.15252/embr.202256702},
url = {https://pub.dzne.de/record/263802},
}