Table 1.

Summary of Investigated Markers

	Rostral cortex sections		Cortical neuron cultures
	Similar staining patterns in MPSIIIB and wild type	Colocalization with LAMP1 in MPSIIIB*	Similar staining patterns in MPSIIIB and wild type	More frequent colocalization with LAMP1 in MPSIIIB than in wild type†
ER markers
Calnexin	ND	ND	Yes	No
ER-to-Golgi markers
ERGIC53	Yes	No	Yes	No
COPII (sec23)	Yes	Yes‡	Yes	Yes (×1.6)
betaCOP	Yes	No	Yes	No
Golgi markers
GM130	No§	Yes	No§	Yes (×2.3)
Syntaxin 5	ND	ND	Yes¶	No
Giantin	Yes	No	Yes¶	No
p115	Yes	No	Yes¶	No
GRASP65	Yes	No	Yes¶	No
GS15	ND	ND	Yes¶	No
Golgin 97	Yes	No	Yes¶	No (×1.01)
Endocytic markers
EEA1	Yes	No	Yes	No (×0.83)
M6PR	Yes	No	Yes	No
Autophagic markers
LC3	Yes	No	Yes	No (×0.85)
p62/SQSTM1	Yes	No	Yes	No
Other markers
Ubiquitin	No∥	No	Yes	No
ScMAS	No**	No	Yes	No
Heparan sulfate	No††	No	ND	ND
GM3 ganglioside	ND	ND	No‡‡	No
Alpha synuclein	Yes	No	ND	ND
Neurofilament	Yes	No	ND	ND

ND, not done. 

^*Analyses were performed on confocal brain sections. 

^†Colocalizations observed on apotome sections of cultured neurons were confirmed on confocal sections. MPSIIIB to wild-type ratios of doubly positive vesicles are indicated when colocalization was observed. 

^‡Images were not conclusive on brain sections due to high-density punctuated staining. 

^§Broader signal extending to cell processes in MPSIIIB. 

^¶Typical perinuclear Golgi staining, signal intensity and surface were not quantified. 

^∥Dispersed aggregates in MPSIIIB rostral cortex. 

^**Clustered signals in neuronal soma in MPSIIIB rostral cortex lamina II/III. 

^††Diffuse signal in neuronal soma in MPSIIIB rostral cortex. 

^‡‡More abundant signal in MPSIIIB.