TY  - JOUR
AU  - Ehninger, Dan
AU  - Silva, Alcino J
TI  - Increased levels of anxiety-related behaviors in a Tsc2 dominant negative transgenic mouse model of tuberous sclerosis.
JO  - Behavior genetics
VL  - 41
IS  - 3
SN  - 0001-8244
CY  - Dordrecht [u.a.]
PB  - Springer Science + Business Media B.V
M1  - DZNE-2020-02563
SP  - 357-363
PY  - 2011
AB  - Tuberous sclerosis (TSC) is a single-gene disorder caused by heterozygous mutations in the TSC1 or TSC2 gene. TSC is often associated with neurological (e.g., epilepsy), cognitive (intellectual disabilities, specific neuropsychological impairments) and behavioral pathologies (e.g., autism, attention deficit hyperactivity disorder). In addition, there is a high prevalence of psychiatric problems in TSC populations, including anxiety and mood disorders. To date, little is known about the pathogenetic bases of these associated psychiatric symptoms; for instance, it is unclear whether they are rooted in TSC-associated neurobiological alterations or whether they are secondary psychological phenomena (e.g., because individuals have to cope with the burden of the disease). Here, we report elevated levels of anxiety-related behaviors and mild deficits in two hippocampal-dependent learning tasks in a Tsc2 dominant negative transgenic mouse model of TSC. These findings establish a mouse model for TSC-related anxiety phenotypes and suggest that anxiety disorders in TSC have a biological foundation.
KW  - Animals
KW  - Anxiety: genetics
KW  - Disease Models, Animal
KW  - Female
KW  - Genes, Dominant: genetics
KW  - Genetic Carrier Screening
KW  - Hippocampus: physiopathology
KW  - Humans
KW  - Learning Disabilities: genetics
KW  - Learning Disabilities: physiopathology
KW  - Male
KW  - Memory Disorders: genetics
KW  - Memory Disorders: physiopathology
KW  - Mice
KW  - Mice, Inbred C57BL
KW  - Mice, Transgenic
KW  - Phenotype
KW  - Tuberous Sclerosis: genetics
KW  - Tuberous Sclerosis: physiopathology
KW  - Tuberous Sclerosis Complex 2 Protein
KW  - Tumor Suppressor Proteins: genetics
KW  - TSC2 protein, human (NLM Chemicals)
KW  - Tsc2 protein, mouse (NLM Chemicals)
KW  - Tuberous Sclerosis Complex 2 Protein (NLM Chemicals)
KW  - Tumor Suppressor Proteins (NLM Chemicals)
LB  - PUB:(DE-HGF)16
C6  - pmid:20882401
C2  - pmc:PMC3102774
DO  - DOI:10.1007/s10519-010-9398-1
UR  - https://pub.dzne.de/record/136241
ER  -