The Behavioral profile of severe mental retardation in a genetic mouse model of phenylketonuria

被引:50
作者
Cabib, S
Pascucci, T
Ventura, R
Romano, V
Puglisi-Allegra, S
机构
[1] Univ Roma La Sapienza, Dipartimento Psicol, I-00195 Rome, Italy
[2] Fdn Santa Lucia, IRCCS, Rome, Italy
[3] CNR, Ist Psicobiol & Psicofarmacol, I-00198 Rome, Italy
[4] Univ Palermo, Dipartimento Biopatol & Tecnol Biomed, I-90133 Palermo, Italy
关键词
anxiety; hyperphenylalaninemia; neurodevelopment; object recognition; spatial novelty;
D O I
10.1023/A:1023498508987
中图分类号
B84 [心理学]; C [社会科学总论]; Q98 [人类学];
学科分类号
03 ; 0303 ; 030303 ; 04 ; 0402 ;
摘要
Pah(enu2) mice, created by chemically induced genetic mutation, are characterized by biochemical phenotypes closely resembling untreated human phenylketonuria (PKU). However, studies conducted in adult Pah(enu2) mice have shown no indices of the severe mental retardation that characterizes untreated PKU. The present experiments explored recognition of novel spatial and nonspatial information in Pah(enu2) mice by two nonassociative tests that do not use explicit reinforcement and avoid lengthy training. Moreover, we evaluated emotional reactivity by the Elevated Plus Maze. Finally, the performance of affected mutants was compared with that of their unaffected and heterozygous littermates and also with that of mice of the C57BL/6 (C57) inbred strain, an increasingly used background for genetic targeted organisms, and with DBA/2 (DBA) mice, known for their nonpathological deficits in spatial learning. The results demonstrated that mutant Pah(enu2) mice are characterized by deficits involving both spatial and nonspatial recognition, that are not related to motor impairment or to high emotional reactivity to novelty. These results indicate that Pah(enu2) mice show pathological cognitive deficits and support their use to test hypotheses about neurodevelopmental disturbances involved in mental retardation.
引用
收藏
页码:301 / 310
页数:10
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