Table 3.

Microglial activation and astrocytic GFAP expression in brains of LSD model mice.

	Iba1				GFAP
	HC	CB	CX	BS	HC	CB	CX	BS
Gaucher	nc	nc	nc	nc	0	0	0	0
Fabry	nc	nc	nc	nc	0	0	0	0
Sandhoff	+	+	+	+	0	100	60	0
NPA	++	++	++	++	0	100	0	0
Hurler	+	+	+	+	0	0	0	0
Pompe	++	++	++	++	20	80	0	0
NPC	+++	+++	+++	+++	0	60	0	0

Iba1 staining was assessed and compared to wild-type controls. GFAP immunoreactivity is quantified as percent of mice exhibiting increased signal in a particular brain area (n = 5/group, except Gaucher mouse model which was compared to historical controls (Sardi et al., 2011; Sardi et al., 2013). See Methods for experimental details regarding staining and blinded semiquantitative assessment. Abbreviations: Iba1: nc = no change compared to wild-type controls, + = mild activation, ++ = moderate activation, +++ = severe activation. GFAP: numbers show percentage of mice evaluated, which demonstrated increased astrogliosis.