Neurobehavioral Alterations in a Genetic Murine Model of Feingold Syndrome 2

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...

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Published in:	Behavior genetics Vol. 45; no. 5; pp. 547 - 559
Main Authors:	Fiori, E., Babicola, L., Andolina, D., Coassin, A., Pascucci, T., Patella, L., Han, Y.-C., Ventura, A., Ventura, R.
Format:	Journal Article
Language:	English
Published:	New York Springer US 01-09-2015 Springer Nature B.V
Subjects:	Animals Apoptosis Behavior, Animal - physiology Behavioral Science and Psychology Brain - physiopathology Brain research Cell cycle Clinical Psychology Disease Models, Animal Dopamine Esophagus Eyelids - abnormalities Eyelids - physiopathology Female Health Psychology Intellectual Disability - complications Intellectual Disability - genetics Intellectual Disability - physiopathology Learning disabilities Limb Deformities, Congenital - complications Limb Deformities, Congenital - genetics Limb Deformities, Congenital - physiopathology Male Mental Disorders - genetics Mice Mice, Inbred C57BL Microcephaly Microcephaly - complications Microcephaly - genetics Microcephaly - physiopathology MicroRNAs MicroRNAs - genetics Original Research Psychology Public Health Tracheoesophageal Fistula - complications Tracheoesophageal Fistula - genetics Tracheoesophageal Fistula - physiopathology New York Italy United States > US Rome Italy Animal model Behavior Cognitive deficit Feingold 2 syndrome
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Summary:	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.
Bibliography:	ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23 E. Fiori and L. Babicola have contributed equally to this work.
ISSN:	0001-8244 1573-3297
DOI:	10.1007/s10519-015-9724-8