Albini, Martina;
Almacellas-Barbanoj, Amanda;
Krawczun-Rygmaczewska, Alicja;
Ciano, Lorenzo;
Benfenati, Fabio;
Michetti, Caterina;
Cesca, Fabrizia;
(2024)
Alterations in KIDINS220/ARMS Expression Impact Sensory Processing and Social Behavior in Adult Mice.
International Journal of Molecular Sciences
, 25
(4)
, Article 2334. 10.3390/ijms25042334.
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(Albni, M and Almacellas-Barbanoj et al., 2024)Alterations in KIDINS220 ARMS Expression Impact Sensory processing and social behavior in adult mice.pdf - Published Version Download (1MB) | Preview |
Abstract
Kinase D-interacting substrate of 220 kDa (Kidins220) is a transmembrane protein that participates in neural cell survival, maturation, and plasticity. Mutations in the human KIDINS220 gene are associated with a neurodevelopmental disorder (‘SINO’ syndrome) characterized by spastic paraplegia, intellectual disability, and in some cases, autism spectrum disorder. To better understand the pathophysiology of KIDINS220-linked pathologies, in this study, we assessed the sensory processing and social behavior of transgenic mouse lines with reduced Kidins220 expression: the CaMKII-driven conditional knockout (cKO) line, lacking Kidins220 in adult forebrain excitatory neurons, and the Kidins220floxed line, expressing constitutively lower protein levels. We show that alterations in Kidins220 expression levels and its splicing pattern cause impaired response to both auditory and olfactory stimuli. Both transgenic lines show impaired startle response to high intensity sounds, with preserved pre-pulsed inhibition, and strongly reduced social odor recognition. In the Kidins220floxed line, olfactory alterations are associated with deficits in social memory and increased aggressive behavior. Our results broaden our knowledge of the SINO syndrome; understanding sensory information processing and its deviations under neuropathological conditions is crucial for devising future therapeutic strategies to enhance the quality of life of affected individuals.
| Type: | Article |
|---|---|
| Title: | Alterations in KIDINS220/ARMS Expression Impact Sensory Processing and Social Behavior in Adult Mice |
| Location: | Switzerland |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.3390/ijms25042334 |
| Publisher version: | https://doi.org/10.3390/ijms25042334 |
| Language: | English |
| Additional information: | © 2024 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). |
| Keywords: | KIDINS220/ARMS; SINO syndrome; transgenic mouse lines; sensory processing; sensory–motor gating; social behavior; aggressive behavior |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Clinical and Experimental Epilepsy |
| URI: | https://discovery.ucl.ac.uk/id/eprint/10190165 |
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