2011
DOI: 10.1186/1471-2156-12-7
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Normal social seeking behavior, hypoactivity and reduced exploratory range in a mouse model of Angelman syndrome

Abstract: BackgroundAngelman syndrome (AS) is a neurogenetic disorder characterized by severe developmental delay with mental retardation, a generally happy disposition, ataxia and characteristic behaviors such as inappropriate laughter, social-seeking behavior and hyperactivity. The majority of AS cases are due to loss of the maternal copy of the UBE3A gene. Maternal Ube3a deficiency (Ube3am-/p+), as well as complete loss of Ube3a expression (Ube3am-/p-), have been reproduced in the mouse model used here.ResultsHere we… Show more

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Cited by 35 publications
(42 citation statements)
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“…Therefore, we used the total activity in the first 15 minutes following injection to compare cocaine-stimulated locomotor activity between genotypes. Total locomotion over the 15 minutes following saline injection was lower in Ube3a m-/p+ mice (531 ± 51 cm) than in WT mice (916 ± 79 cm; U = 266, P < 0.001), consistent with decreased motor activity previously described in Ube3a m-/p+ mice (20). Although a low cocaine dose (5.6 mg/kg; Figure 3A) was sufficient in both genotypes to induce locomotor sensitization, defined as greater distance traveled on the challenge/last day compared with that on the first day of administration (P < 0.001), we found Ube3a m-/p+ mice to be less sensitized than WT mice (day 4, P = 0.02; day 5, P = 0.031; challenge, P = 0.031; Figure 3D).…”
Section: Figuresupporting
confidence: 65%
See 1 more Smart Citation
“…Therefore, we used the total activity in the first 15 minutes following injection to compare cocaine-stimulated locomotor activity between genotypes. Total locomotion over the 15 minutes following saline injection was lower in Ube3a m-/p+ mice (531 ± 51 cm) than in WT mice (916 ± 79 cm; U = 266, P < 0.001), consistent with decreased motor activity previously described in Ube3a m-/p+ mice (20). Although a low cocaine dose (5.6 mg/kg; Figure 3A) was sufficient in both genotypes to induce locomotor sensitization, defined as greater distance traveled on the challenge/last day compared with that on the first day of administration (P < 0.001), we found Ube3a m-/p+ mice to be less sensitized than WT mice (day 4, P = 0.02; day 5, P = 0.031; challenge, P = 0.031; Figure 3D).…”
Section: Figuresupporting
confidence: 65%
“…(D) The maximum rate of operant responding for rewarding brain stimulation is comparable between genotypes (P > 0.05). (E) Ube3a m-/p+ mice maintain a lower reward threshold over time (16)(17)(18)(19)(20)(21)(22)(23)(24)(25)(26)(27)(28)(29)(30) minutes, ***P < 0.001; 31-45 minutes, ***P < 0.001; 46-60 minutes, *P = 0.026). (F) WT mice exhibit greater potentiation of rewarding brain stimulation expressed as lower reward thresholds than Ube3a m-/p+ mice following 10.0 mg/kg (**P = 0.002) and 17.0 mg/kg (***P < 0.001) GBR 12909 (i.p.).…”
Section: Figurementioning
confidence: 99%
“…However, no IGs responsible for this behavior have yet been discovered although notably Peg3 KO females are more maternally aggressive [26]. (v) Mice possessing a paternal duplication of a region of mouse chromosome 7 (orthologous to human chromosome 15q11-13) show reduced social investigation behavior when given access to a novel stranger mouse, suggesting that MEGs promote social interaction [25], though mice lacking the MEG Ube3a which is contained within that genomic region show no differences in this behavior [71].…”
Section: Box 1 Igs and Mouse Social Behaviormentioning
confidence: 96%
“…They also display various motor defects [71], abnormal cerebellum-driven licking behavior [72], sleep disturbance [73], abnormal EEG patterns, and cognitive defects in the conditioned fear and Morris water maze tests [74]. However, these mice displayed hypoactivity and normal social seeking behavior, in contrast to what is seen in human patients with AS and/or ASD [75]. Importantly, human patients with mutations in UBE3A typically have a milder phenotype than those patients harboring interstitial deletions, so this mouse model may not accurately reflect the majority of AS patients, who have a deletion containing this gene, as well as many other genes at this locus.…”
Section: Modeling 15q11-13 Cnvs In Micementioning
confidence: 83%