Table 3. Potential CSF Biomarkers in CLN2^a.

gene name	ENSEMBL protein ID	CSF ratio compared with control	brain ratio compared with control
MB	ENSP00000380489	5.19
ALDH1A2*	ENSP00000249750	4.80
HBG2	ENSP00000369609	4.75	0.59
FBP1	ENSP00000364475	4.71
VIM*	ENSP00000224237	4.49	1.49
HBB*	ENSP00000333994	4.24	0.81
CALB1	ENSP00000265431	4.09	1.30
CRABP2	ENSP00000357205	4.03
CRABP1*	ENSP00000299529	3.84	ns
FLNC	ENSP00000327145	3.42	1.62
PON1	ENSP00000222381	3.34
APOB*	ENSP00000233242	3.25	ns
CA1	ENSP00000430656	3.17	0.70
EEA1	ENSP00000317955	2.99	0.82
HBD	ENSP00000369654	2.96	ns
HBA2	ENSP00000251595	2.93	0.84
KCTD12	ENSP00000366694	2.84	ns
F13A1*	ENSP00000264870	2.81	ns
ANXA1	ENSP00000257497	2.72	ns
ACOT1	ENSP00000311224	2.68	ns
HSPD1	ENSP00000373620	2.61	0.82
COL14A1*	ENSP00000311809	2.61	ns
S100A11	ENSP00000271638	2.56	ns
TIMP2	ENSP00000262768	0.39
KLK6*	ENSP00000366047	0.39
NELL2	ENSP00000390680	0.39	ns
TPPP	ENSP00000353785	0.38	0.59
HAPLN2	ENSP00000255039	0.32	0.55
CBLN3	ENSP00000267406	0.32
MAG*	ENSP00000376048	0.29	1.45
TPP1	ENSP00000299427	0.28	0.39
CNDP1*	ENSP00000351682	0.27	ns
ENSP00000457200*	ENSP00000457200	0.25	1.12
PMP2	ENSP00000256103	0.25	0.61

^aProteins that are significantly elevated or reduced in CSF by more than 2.5-fold are shown, together with respective brain fold-changes (blank indicates not found in brain; ns, no significant difference between NCL and control). Data are expressed as fold-change compared with average control. Data in bold indicate proteins for which changes in expression between brain and CSF are not consistent.

Asterisks indicate proteins that are significantly altered in all three NCLs in the same direction.