Skip to main content
. Author manuscript; available in PMC: 2023 Sep 2.
Published in final edited form as: Neurobiol Dis. 2022 Sep 14;174:105862. doi: 10.1016/j.nbd.2022.105862

Fig. 1.

Fig. 1.

Biochemical abnormalities in the periventricular white matter of KD. Homogenates of periventricular white matter (PVWM) from 4 KD and 4 age-matched normal control cases are analyzed for lysosomal GALC protein (Lys-GALC), mature β-subunit of acid ceramidase (ASAH1), myelin basic protein (MBP), lysosome-associated membrane protein 1 (LAMP1) and glyceraldehyde-3-phosphate dehydrogenase (GAPDH) by Western blotting (a). Protein levels are quantified by densitometric measurement of target band intensity normalized to GAPDH levels. Quantification data of Lys-GALC, ASAH1, MBP, LAMP1 and hyperglycosylated LAMP1 (hi-LAMP1) are shown in panel b, f, g, h and i, respectively. The same samples are also analyzed for GALC activity (c), total GALC protein levels (d), and psychosine levels (e). Increased psychosine levels (fold change) in the three infantile KD cases compared to age-matched controls are also annotated (e). Error bars represent standard error mean of triplicate samples.