Table 1.

Neurologic features in Tsc1^null-neuron mice according to age and treatment

	Genotype class	Treatment	Age	n	Weight (g)	Weight SD	Clasp	Tremor	Kyphosis	Tail
% Tsc1^c− SynICre⁺
46%	Mutant	None	P30	16	9.87	1.59	0.63	3.28	0.81	2.73
65%	Mutant	Rapamycin	P30	17	10.27	2.05	0	0.12	0	0.88
88%	Mutant	RAD001	P30	17	11.14	1.14	0	0.26	0	1.24
92%	Mutant	Rapamycin	P60	12	14.39	2.68	0	0.42	0.17	0.83
86%	Mutant	rap-P30	P60	9	16.13	2.48	0	1.94	0.67	1.14
71%	Mutant	Rapamycin	P100	14	18.13	1.84	0	0.64	0.14	0.79
82%	Mutant	RAD001	P100	11	19.59	2.30	0.09	1.00	0.27	0.73
% Tsc1^c+ SynICre⁺
89%	Control	None	P30	10	20.32	2.85	0	0.35	0	0.30
100%	Control	Rapamycin	P30	4	11.50	0.27	0	0.25	0	0
86%	Control	RAD001	P30	7	12.66	2.33	0.14	0.14	0	0.14
57%	Control	Rapamycin	P60	7	18.39	2.31	0	0.36	0	0.57
75%	Control	Rapamycin	P100	9	19.56	2.25	0	0.39	0	0.56
78%	Control	RAD001	P100	9	20.76	2.91	0	0.22	0.22	0.44

Mutant refers to Tsc1^null-neuron mice. Mutants had genotypes Tsc1^c− SynICre⁺ or Tsc1^cc SynICre⁺; % Tsc1^c− SynICre⁺ is shown in the first column for mutants. Controls had genotypes Tsc1^c+ SynICre⁺, Tsc1^c+ SynICre⁻, Tsc1^cc SynICre⁻, Tsc1^c− SynICre⁻; % Tsc1^c+ SynICre⁺ is shown in the first column for controls. All treatments were 6 mg/kg intraperitoneally every other day, beginning at P7–P9. Rapamycin on/off mice were treated until P30, and then drug was discontinued; all others were treated continuously. Average values for weight in grams and standard deviation (SD) are shown. Average values for clasping, tremor, kyphosis, and tail position scores are shown and were assessed as described in Materials and Methods. Tremor and tail position were scored on 0–5 and 0–3 scales, respectively. Clasping and kyphosis were assessed on a binary 0 (absent) or 1 (present) scale. Note that all neurologic scores as well as weight were significantly different (p < 0.003 for each) between untreated mutant and control mice at P30. All neurologic scores, but not weight, were also significantly different (p < 0.003 for each) between P30 mutant and rapamcyin- or RAD001-treated P30 mice. There was no significant difference between weight or any neurologic score between rapamycin- and RAD001- treated mice at either P30 or P100. There was a significant difference between rapamycin-treated P60 and rapamycin-treated 22 d only P60 (rap on/off) mice in both tremor (p = 0.0003) and kyphosis (p = 0.02).