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-17–92D/? 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-17–92D/? 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-17–92D/? 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-17–92D/? 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-17–92D/? in comparison with WT mice, thus suggesting a possible link between cognitive deficits and altered brain neurotransmission.

Neurobehavioral alterations in a genetic murine model of Feingold syndrome 2 / Fiori, Elena; Babicola, Lucy; Andolina, Diego; Coassin, A.; Pascucci, Tiziana; Patella, Loris; Han, Y. C.; Ventura, A.; Ventura, Rossella. - In: BEHAVIOR GENETICS. - ISSN 0001-8244. - ELETTRONICO. - 45:5(2015), pp. 547-559. [10.1007/s10519-015-9724-8]

Neurobehavioral alterations in a genetic murine model of Feingold syndrome 2

Fiori, Elena;BABICOLA, LUCY;ANDOLINA, DIEGO;PASCUCCI, Tiziana;Patella, Loris;VENTURA, Rossella
Writing – Original Draft Preparation
2015

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-17–92D/? 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-17–92D/? 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-17–92D/? 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-17–92D/? 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-17–92D/? in comparison with WT mice, thus suggesting a possible link between cognitive deficits and altered brain neurotransmission.
2015
animal model; behavior; cognitive deficit; Feingold 2 syndrome; genetics
01 Pubblicazione su rivista::01a Articolo in rivista
Neurobehavioral alterations in a genetic murine model of Feingold syndrome 2 / Fiori, Elena; Babicola, Lucy; Andolina, Diego; Coassin, A.; Pascucci, Tiziana; Patella, Loris; Han, Y. C.; Ventura, A.; Ventura, Rossella. - In: BEHAVIOR GENETICS. - ISSN 0001-8244. - ELETTRONICO. - 45:5(2015), pp. 547-559. [10.1007/s10519-015-9724-8]
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/793862
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