Lysosome function, as assessed by Lamp1 levels, are unchanged in the GBA1 D409V KI mouse model.

(A-B) Lamp1 levels were measured by Western blot in brain and liver homogenate from C57Bl/6 wild type (WT) mice and GBA1 D409V KI homozygous (HOM) mice at 4, 8, and 12 months of age (n = 7/group). Levels of Lamp1 were not significantly different between WT and GBA1 D409V KI mice at any age in brain (A) or liver (B) tissue. There is an age-related increase in Lamp1 appearing at 12 months of age, with statistically significant differences in the brain and liver of GBA1 D409V KI mice and the liver of WT mice. Lamp1 protein levels are normalized to β-actin as the housekeeping protein. Brain LAMP1 (A) measured as follows: WT 4mo 103.6±3.35, HOM 4mo 98.95±4.40, WT 8mo 124.8±3.11, HOM 8mo 130.7±3.40, WT 12 mo 93.27±2.14, HOM 12mo 121.80±1.98. Liver LAMP1 (B) measured as follows: WT 4mo 0.35±0.02, HOM 4mo 0.37±0.02, WT 8mo 0.30±0.02, HOM 8mo 0.32±0.02, WT 12 mo 0.33±0.01, HOM 12mo 0.48±0.04. Abbreviations: LAMP1, lysosomal associated membrane protein 1; GBA1, gene encoding glucocerebrosidase; WT, wild type; HOM, homozygous; KI, knockin; SEM, standard error of the mean; mo, month; ns, non-significant (p > 0.05).

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