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Web End = Behav Genet (2015) 45:547559 DOI 10.1007/s10519-015-9724-8
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Web End = Neurobehavioral Alterations in a Genetic Murine Model of Feingold Syndrome 2
E. Fiori1,2 L. Babicola2,3 D. Andolina2,3 A. Coassin1,2
T. Pascucci1,2 L. Patella2,3 Y.-C. Han4 A. Ventura5
R. Ventura1,2
Received: 19 September 2014 / Accepted: 20 May 2015 / Published online: 31 May 2015 Springer Science+Business Media New York 2015
Abstract Feingold syndrome (FS) is an autosomal dominant disorder characterized by microcephaly, short stature, digital anomalies, esophageal/duodenal atresia, facial dys-morphism, and various learning disabilities. Heterozygous deletion of the miR-1792 cluster is responsible for a subset of FS (Feingold syndrome type 2, FS2), and the developmental abnormalities that characterize this disorder are partially recapitulated in mice that harbor a heterozygous deletion of this cluster (miR-1792D/? mice). Although
Feingold patients develop a wide array of learning disabilities, no scientic description of learning/cognitive disabilities, intellectual deciency, and brain alterations have been described in humans and animal models of FS2. The aim of this study was to draw a behavioral prole, during development and in adulthood, of miR-1792D/? mice, a genetic
mouse model of FS2. Moreover, dopamine, norepinephrine and serotonin tissue levels in the medial prefrontal cortex (mpFC), and Hippocampus (Hip) of miR-1792D/? mice were analyzed.Our data showed decreased body growth and reduced vocalization during development. Moreover, selective decits in spatial ability, social novelty recognition and memory span were evident in adult miR-1792D/? mice compared with healthy controls (WT). Finally, we found altered dopamine as well as serotonin tissue levels, in the mpFC and Hip, respectively, of miR-1792D/? in comparison with WT mice, thus suggesting a possible link between cognitive decits and altered brain neurotransmission.
Keywords Animal model Feingold 2 syndrome
Behavior Cognitive decit
Introduction
MicroRNAs (miRNAs) are small noncoding RNAs, approximately 21 nucleotides in length, that regulate gene expression at the post-transcriptional level by inducing mRNA destabilization and translational inhibition of target mRNAs (Baek et al. 2008; Bartel 2004; Selbach et al. 2008). Initially described as modulators of developmental timing in Caenorhabditis elegans (Lee et al. 1993; Wightman et al....