Neurobehavioral Alterations in a Genetic Murine Model of Feingold Syndrome 2.
Neurobehavioral Alterations in a Genetic Murine Model of Feingold Syndrome 2.
复制标题
Feingold 综合征 2 遗传小鼠模型中的神经行为改变。
DOI：
10.1007/s10519-015-9724-8
复制
发表时间：
2015
影响因子：
2.6
通讯作者：
Ventura,R
中科院分区：
文献类型：
--
作者：
Fiori,E;Babicola,L;Andolina,D;Coassin,A;Pascucci,T;Patella,L;Han,Y-C;Ventura,A;Ventura,R
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 themiR-17–92cluster 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.
