Neurobehavioral Alterations in a Genetic Murine Model of Feingold Syndrome 2

E. Fiori, L. Babicola, D. Andolina, A. Coassin, T. Pascucci, L. Patella, Y. C. Han, A. Ventura, R. Ventura

Research output: Contribution to journalArticle

Abstract

Feingold syndrome (FS) is an autosomal dominant disorder characterized by microcephaly, short stature, digital anomalies, esophageal/duodenal atresia, facial dysmorphism, and various learning disabilities. Heterozygous deletion of the miR-17–92 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-17–92∆/+ mice). Although Feingold patients develop a wide array of learning disabilities, no scientific description of learning/cognitive disabilities, intellectual deficiency, and brain alterations have been described in humans and animal models of FS2. The aim of this study was to draw a behavioral profile, during development and in adulthood, of miR-17–92∆/+ 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-17–92∆/+ mice were analyzed.Our data showed decreased body growth and reduced vocalization during development. Moreover, selective deficits in spatial ability, social novelty recognition and memory span were evident in adult miR-17–92∆/+ 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-17–92∆/+ in comparison with WT mice, thus suggesting a possible link between cognitive deficits and altered brain neurotransmission.

Original languageEnglish
Pages (from-to)547-559
Number of pages13
JournalBehavior Genetics
Volume45
Issue number5
DOIs
Publication statusPublished - May 31 2015

Keywords

  • Animal model
  • Behavior
  • Cognitive deficit
  • Feingold 2 syndrome

ASJC Scopus subject areas

  • Genetics
  • Genetics(clinical)
  • Ecology, Evolution, Behavior and Systematics

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    Fiori, E., Babicola, L., Andolina, D., Coassin, A., Pascucci, T., Patella, L., Han, Y. C., Ventura, A., & Ventura, R. (2015). Neurobehavioral Alterations in a Genetic Murine Model of Feingold Syndrome 2. Behavior Genetics, 45(5), 547-559. https://doi.org/10.1007/s10519-015-9724-8