Table 1.

Similarities between gabrb3^−/− mice and Angelman syndrome

Characteristic	gabrb3^−/−mice	Angelman syndrome
Facial dysmorphism	Cleft Palate^b	Large mouth, widely spaced teeth^c
Poor learning and memory	+^a	Mental retardation^c
Poor motor coordination	+^a	Ataxia, unsteady gait^c
Abnormal rest-activity pattern	+^a	+^c
Hyperactivity	+^a	+^d
Repetitive, stereotypical behavior	+^a	+^d
Hyperresponsive	Hypersensitive^b	Hyperreflexive^e
Neonatal feeding problems	+^b	+^c
Reduction in benzodiazepine binding in brain	+^b	+^f
Epilepsy
Age-dependent epileptic evolution	+^a	+^g
Myoclonic jerks	+^a	+^h
Multiple seizure types	+^a	+ⁱ
Worsens with carbamazepine	+^a	+^e,i
Ethosuximide reduces ictal occurrence	+^a	+^j
EEG
Prominent high amplitude polymorphic slow wave discharges	+^a	+ⁱ
Generalized ictal discharges	+^a	+^e
EEG worsens on eye closure/drowsiness	+^a	+^k
High amplitude epileptic spikes	+^a	+^k
Intermittent photic stimulation had no effect	+^a	+^k

Clinical characteristics of Angelman syndrome and features observed in gabrb3^−/− knock-out mice are from the following citations: ^aThis paper,^bHomanics et al., 1997;^cWilliams et al., 1995;^dSummers et al., 1995;^eViani et al., 1995;^fOdano et al., 1996;^gMatsumoto et al., 1992;^hGuerrini et al., 1996;ⁱMinassian et al., 1998;^jLaan et al., 1996; ^kBoyd et al., 1988.