Table 2 Pearson correlation coefficients and p-values of CLN2 signature metabolite abundances (normalized) and the MRIDSS, WCLS or age for all individuals (test and validation cohort).

Identification	Metabolite feature	WCLS		MRIDSS		Age (Months)
Identification	Metabolite feature	Pearson r	p-value	Pearson r	p-value	Pearson r	p-value
NAAG	Unknown 1	0.74	<0.0001	0.76	<0.0001	−0.7215	0.0002
NAAG-Adduct	Unknown 1 Adduct	0.75	<0.0001	0.78	<0.0001	0.1565	0.1976
Glycero-3-phosphoinositol	Unknown 2	0.6	0.003	0.57	0.0056	−0.5017	0.0174
NANA	N-Acetylneuraminic Acid	0.65	0.0009	0.59	0.0036	−0.3841	0.0776
NANA Dimer	Unknown 3	0.74	<0.0001	0.7	0.0003	−0.4997	0.0179
139.981	Unknown 4 parent	0.65	0.001	0.6	0.0029	−0.3848	0.077
95.988	Unknown 4	0.64	0.0015	0.57	0.006	−0.4181	0.0528
N-Acetylalanine	Unknown 5	0.66	0.0008	0.65	0.001	−0.5332	0.0106
N-Acetylglutamic Acid		0.11	0.6278	0.01	0.9585	−0.2377	0.2867
N-Acetylglycine		0.48	0.0224	0.28	0.2037	−0.37	0.0901
N-Acetylserine		0.67	0.0007	0.72	0.0001	−0.4748	0.0256
N-Acetylthreonine		0.55	0.0079	0.5	0.0175	−0.4243	0.0491

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