Figure 2: Lysosomal expansion but not astrocytosis and ganglioside accumulation are present in the developing postnatal primary somatosensory cortex.
(A) Representative confocal images from unaffected (Sgsh+/h*) and hypomorphic MPS IIIA (Sgshh/h) mouse primary somatosensory cortex stained with LAMP-1 (green). Left panels show low magnification images, scale bar 100 μm. Right panels show high magnification of layer II/III (boxed region), scale bar 50 μm. Insets in lower right corner show single cells. Nuclei were stained with DAPI (blue). (B) Quantification of LAMP-1 stained vesicle area from high magnification images. (C) Representative confocal images stained with a marker for reactive cortical astrocytes, GFAP (red) and ganglioside GM3 (green), as described in (A). Arrows in low magnification images point to cells with intracellular staining of GM3, denoted by asterisks in high magnification images. P, pial basement membrane; WM, white matter; BV, blood vessel. (D) Quantification of GFAP immunoreactivity in the parenchyma of the somatosensory cortex. (E) Quantification of GM3 immunoreactivity in the parenchyma of the somatosensory cortex. N = 3 animals per genotype per age, 2 sections per animal, 2 regions of interest per section were quantified. All brains were collected, stained, and analyzed at the same time. Findings were confirmed on a separate cohort of N = 3 animals per genotype per age. Mean ± SD.
