scholarly journals Over-expression of RCAN1 causes Down syndrome-like hippocampal deficits that alter learning and memory

2012 ◽  
Vol 21 (13) ◽  
pp. 3025-3041 ◽  
Author(s):  
Katherine R. Martin ◽  
Alicia Corlett ◽  
Daphne Dubach ◽  
Tomris Mustafa ◽  
Harold A. Coleman ◽  
...  
Keyword(s):  
Down Syndrome ◽  
Learning And Memory ◽  
Over Expression

scholarly journals Dosage-dependent over-expression of genes in the trisomic region of Ts1Cje mouse model for Down syndrome

2004 ◽  
Vol 13 (13) ◽  
pp. 1333-1340 ◽  
Author(s):  
Kenji Amano ◽  
Haruhiko Sago ◽  
Chiharu Uchikawa ◽  
Taishi Suzuki ◽  
Svetlana E. Kotliarova ◽  
...  
Keyword(s):  
Down Syndrome ◽  
Mouse Model ◽  
Over Expression ◽  
Expression Of Genes

Over-expression of two different forms of the α-secretase ADAM10 affects learning and memory in mice

2006 ◽  
Vol 175 (2) ◽  
pp. 278-284 ◽  
Author(s):  
Ulrich Schmitt ◽  
Christoph Hiemke ◽  
Falk Fahrenholz ◽  
Anja Schroeder
Keyword(s):  
Learning And Memory ◽  
Over Expression

Implicit and explicit memory functioning in children with heavy prenatal alcohol exposure

1999 ◽  
Vol 5 (5) ◽  
pp. 462-471 ◽  
Author(s):  
SARAH N. MATTSON ◽  
EDWARD P. RILEY
Keyword(s):  
Down Syndrome ◽  
Free Recall ◽  
Recognition Memory ◽  
Learning And Memory ◽  
Prenatal Alcohol Exposure ◽  
Alcohol Exposure ◽  
Memory Deficits ◽  
Children With Down Syndrome ◽  
Memory Functioning ◽  
Prenatal Alcohol

Prenatal alcohol exposure is associated with widespread and devastating neurodevelopmental deficits. Numerous reports have suggested memory deficits in both humans and animals exposed prenatally to alcohol. However, the nature of these memory deficits remains to be characterized. Recently children with fetal alcohol syndrome were shown to have learning and memory deficits on a verbal learning and memory measure that involved free recall and recognition memory. The current study seeks to further characterize memory functioning in children with heavy prenatal alcohol exposure by evaluating priming performance. The choice of task is also relevant given previous studies of memory performance in patient groups with and without involvement of the basal ganglia, a group of structures known to be affected in fetal alcohol syndrome. Three groups were evaluated for lexical priming, free recall, recognition memory, and verbal fluency: (1) children with heavy prenatal alcohol exposure; (2) children with Down syndrome; and (3) nonexposed controls. The children with Down syndrome showed significantly less priming than alcohol-exposed children, who did not differ from controls. In addition, the alcohol-exposed children were impaired on the free recall task but not on the recognition memory task, whereas the children with Down syndrome performed significantly worse than the alcohol-exposed group on both tasks. Finally, on the verbal fluency task, children with heavy prenatal alcohol exposure were impaired on both category and letter fluency, but the degree of impairment was greater for letter fluency. These results further characterize the memory deficits in children with heavy prenatal alcohol exposure suggesting that in spite of learning and memory deficits, they are able to benefit from priming of verbal information. (JINS, 1999, 5, 462–471.)

scholarly journals Early-occurring proliferation defects in peripheral tissues of the Ts65Dn mouse model of Down syndrome are associated with patched1 over expression

2012 ◽  
Vol 92 (11) ◽  
pp. 1648-1660 ◽  
Author(s):  
Claudia Fuchs ◽  
Elisabetta Ciani ◽  
Sandra Guidi ◽  
Stefania Trazzi ◽  
Renata Bartesaghi
Keyword(s):  
Down Syndrome ◽  
Mouse Model ◽  
Ts65dn Mouse ◽  
Peripheral Tissues ◽  
Over Expression

scholarly journals Forebrain Shh overexpression improves cognitive function and locomotor hyperactivity in an aneuploid mouse model of Down syndrome and its euploid littermates

2021 ◽  
Vol 9 (1) ◽  
Author(s):  
Feng J. Gao ◽  
Donna Klinedinst ◽  
Fabian-Xosé Fernandez ◽  
Bei Cheng ◽  
Alena Savonenko ◽  
...  
Keyword(s):  
Down Syndrome ◽  
Cognitive Function ◽  
Mouse Model ◽  
Learning And Memory ◽  
Perinatal Period ◽  
Cerebellar Hypoplasia ◽  
Shh Signaling ◽  
Site Specific ◽  
Locomotor Hyperactivity

AbstractDown syndrome (DS) is the leading genetic cause of intellectual disability and causes early-onset dementia and cerebellar hypoplasia. The prevalence of attention deficit hyperactivity disorder is elevated in children with DS. The aneuploid DS mouse model “Ts65Dn” shows prominent brain phenotypes, including learning and memory deficits, cerebellar hypoplasia, and locomotor hyperactivity. Previous studies indicate that impaired Sonic hedgehog (Shh) signaling contributes to neurological phenotypes associated with DS and neurodegenerative diseases. However, because of a lack of working inducible Shh knock-in mice, brain region-specific Shh overexpression and its effects on cognitive function have not been studied in vivo. Here, with Gli1-LacZ reporter mice, we demonstrated that Ts65Dn had reduced levels of Gli1, a sensitive readout of Shh signaling, in both hippocampus and cerebellum at postnatal day 6. Through site-specific transgenesis, we generated an inducible human Shh knock-in mouse, TRE-bi-hShh-Zsgreen1 (TRE-hShh), simultaneously expressing dually-lipidated Shh-Np and Zsgreen1 marker in the presence of transactivator (tTA). Double transgenic mice “Camk2a-tTA;TRE-hShh” and “Pcp2-tTA;TRE-hShh” induced Shh overexpression and activated Shh signaling in a forebrain and cerebellum, respectively, specific manner from the perinatal period. Camk2a-tTA;TRE-hShh normalized locomotor hyperactivity and improved learning and memory in 3-month-old Ts65Dn, mitigated early-onset severe cognitive impairment in 7-month-old Ts65Dn, and enhanced spatial cognition in euploid mice. Camk2a-tTA;TRE-hShh cohort maintained until 600days old showed that chronic overexpression of Shh in forebrain from the perinatal period had no effect on longevity of euploid or Ts65Dn. Pcp2-tTA;TRE-hShh did not affect cognition but mitigated the phenotype of cerebellar hypoplasia in Ts65Dn. Our study provides the first in vivo evidence that Shh overexpression from the perinatal period protects DS brain integrity and enhances learning and memory in normal mice, indicating the broad therapeutic potential of Shh ligand for other neurological conditions. Moreover, the first inducible hShh site-specific knock-in mouse could be widely used for spatiotemporal Shh signaling regulation.

scholarly journals Rescue of Synaptic Failure and Alleviation of Learning and Memory Impairments in a Trisomic Mouse Model of Down Syndrome

2011 ◽  
Vol 70 (12) ◽  
pp. 1070-1079 ◽  
Author(s):  
Julie Blanchard ◽  
Silvia Bolognin ◽  
Muhammad Omar Chohan ◽  
Ausma Rabe ◽  
Khalid Iqbal ◽  
...  
Keyword(s):  
Down Syndrome ◽  
Mouse Model ◽  
Learning And Memory ◽  
Memory Impairments ◽  
Synaptic Failure

scholarly journals LDOC1 expression in fibroblasts of patients with Down syndrome

2015 ◽  
Vol 10 (1) ◽  
Author(s):  
Michele Salemi ◽  
Concetta Barone ◽  
Carmelo Romano ◽  
Salvatore Caniglia ◽  
Alda Ragalmuto ◽  
...  
Keyword(s):  
Cell Death ◽  
Down Syndrome ◽  
Trisomy 21 ◽  
Leucine Zipper ◽  
Normal Subjects ◽  
Death Process ◽  
Over Expression ◽  
Cell Death Process ◽  
Apoptotic Pathways ◽  
Gene Mrna

AbstractDown syndrome (DS) is characterised by intellectual disability and is caused by trisomy 21. Apoptosis is a programmed cell death process and is involved in neurodegenerative diseases such as Alzheimer. People with DS can develop some traits of Alzheimer disease at an earlier age than subjects without trisomy 21. The leucine zipper, down regulated in cancer 1 (LDOC1) appears to be involved in the apoptotic pathways. The aim of the present work was to detect the presence of intracellular synthesis of LDOC1 protein and LDOC1 mRNA in fibroblast cultures from DS subjects. The western blot shows the presence of LDOC1 protein in fibroblasts of DS subjects but no evidence of LDOC1 protein in fibroblasts of normal subjects. LDOC1 gene mRNA expression is increased in fibroblasts from DS subjects compared to fibroblasts from normal subjects. The data obtained from this study strengthen the hypothesis that the over-expression of LDOC1 gene could play a role in determining the phenotype of individuals with DS but does not exclude that this results from apoptotic mechanisms.

scholarly journals OLIG2 over-expression impairs proliferation of human Down syndrome neural progenitors

2012 ◽  
Vol 21 (10) ◽  
pp. 2330-2340 ◽  
Author(s):  
Jie Lu ◽  
Gewei Lian ◽  
Hui Zhou ◽  
Giuseppe Esposito ◽  
Luca Steardo ◽  
...  
Keyword(s):  
Down Syndrome ◽  
Neural Progenitors ◽  
Over Expression

scholarly journals Allocentric spatial learning and memory deficits in Down syndrome

2015 ◽  
Vol 6 ◽  
Author(s):  
Pamela Banta Lavenex ◽  
Mathilde Bostelmann ◽  
Catherine Brandner ◽  
Floriana Costanzo ◽  
Emilie Fragnière ◽  
...  
Keyword(s):  
Down Syndrome ◽  
Learning And Memory ◽  
Spatial Learning ◽  
Memory Deficits ◽  
Spatial Learning And Memory ◽  
Learning And Memory Deficits
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