Nucleation of protein aggregation kinetics as a basis for genotype-phenotype correlations in polyglutamine diseases

Table 1 Parameters based on the model: In (t_A² - t_E²)^1/2 = In(a) - bx + ε

	HD	SCA1	SCA7	DRPLA	SCA2	SCA6
% patients	98.42	97.74	97.87	96.70	98.58	93.94
t _E ²	215	170	30	0	80	700
intercept (In(α))	6.657	6.249	6.513	11.011	8.377	9.553
± SE	0.1432	0.1516	0.1697	0.7543	0.3160	0.4076
slope (-b)	0.0662	0.0569	0.0658	0.1247	0.1217	0.2567
± SE	0.00316	0.00298	0.00338	0.0116	0.00773	0.0181
F test	437.65	364.59	380.43	114.96	248.33	201.92
(P)	3.23E-61	4.13E-48	1.84E-46	1.58E-17	1.45E-32	1.23E-27
R ²	0.5854	0.6301	0.6764	0.5721	0.6445	0.6234
R² *	0.5321	0.6259	0.6595	0.5721	0.6391	0.6012

% patients = percent of patients comprised the model, SE = standard error, R² = coefficient determination, R² * = R² derived from linear regression analysis of simple logarithmic transformation of age-of-onset without t_E².

ISSN: 1750-1326