UCL Discovery
UCL home » Library Services » Electronic resources » UCL Discovery

Alterations in KIDINS220/ARMS Expression Impact Sensory Processing and Social Behavior in Adult Mice

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. Green open access

[thumbnail of (Albni, M and Almacellas-Barbanoj et al., 2024)Alterations in KIDINS220 ARMS Expression Impact Sensory processing and social behavior in adult mice.pdf]
Preview
Text
(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
Downloads since deposit
13Downloads
Download activity - last month
Download activity - last 12 months
Downloads by country - last 12 months

Archive Staff Only

View Item View Item