Table 3.

Differential anti-HS antibodies binding in mouse brain

Brain cells	LS-path	HS4E4		HS4C3		EV3C3		AO4B08		RB4Ea12
		wt	IIIB	wt	IIIB	wt	IIIB	wt	IIIB	wt	IIIB
Endothelia	±	+++	++++	+	≥ ++	++	≥ +++	++	++++	–	–
Neurons		+	++++	+	≥ +++	–	++++	–	++++	++	++++
CTX	aY	+	e++++	+	d++++	–	d++++	–	d++++	++	++++
TH	bY	+	e++++	+	e++++	–	e++++	–	e++++	++	++++
HP	bY	–	–	–	–	–	–	–	–	++	++++
BS-M	cY	+	d++++	+	f++++	–	e++++	–	e,f++++	++	++++
BS-I	b,cY	+	e+++		e++++		e++++		e,f++++	++	++++
CB-P	cY	+	f+++	+	f++++	–	d++++	–	f++++	++	++++
CB-G	N	–	–	–	–	–	–	–	–	++	++++
WM-glia	n/a	–	–	–	–	–	–	–	–	++	++++

Cryostat brain sections from wt (n = 4) and MPS IIIB (n = 4) mice were assayed for HS domains by immunofluorescence (IF) staining. LS-Path lysosomal storage pathology, CTX cerebral cortex, TH thalamus, HP hippocampus, BS-M ventral medullary retic nuclear of brain stem, BS-I Intermediate reticular nuclei of brain stem, CB-P cerebellum-Purkinje cells, CB-G cerebellum-granular neurons, WM white matters. Lysosomal storage pathology by toluidine blue staining: ±: Majority endothelial cells do not exhibit lysosomal storage pathology; Y exhibiting lysosomal storage pathology, N no visible lysosomal storage pathology.+–++++: relative IF staining intensities observed under a fluorescent microscope, referring the intensity of HS4C3 stained wt endothelia and HS4E4 stained wt brain cells as +

^aFine foamy vesicles

^bSmall vacuoles

^cLarge vacuoles

Anti-HS antibody staining: ^dFine granules

^eSmall granules

^fCoarse granules