Potocki-Lupski syndrome (PTLS; MIM #610883), characterized by neurobehavioral 
abnormalities, intellectual disability and congenital anomalies, is caused by a 
3.7-Mb duplication in 17p11.2. Neurobehavioral studies determined that ∼70-90% 
of PTLS subjects tested positive for autism or autism spectrum disorder (ASD). 
We previously chromosomally engineered a mouse model for PTLS (Dp(11)17/+) with 
a duplication of a 2-Mb genomic interval syntenic to the PTLS region and 
identified consistent behavioral abnormalities in this mouse model. We now 
report extensive phenotyping with behavioral assays established to evaluate core 
and associated autistic-like traits, including tests for social abnormalities, 
ultrasonic vocalizations, perseverative and stereotypic behaviors, anxiety, 
learning and memory deficits and motor defects. Alterations were identified in 
both core and associated ASD-like traits. Rearing this animal model in an 
enriched environment mitigated some, and even rescued selected, neurobehavioral 
abnormalities, suggesting a role for gene-environment interactions in the 
determination of copy number variation-mediated autism severity.