In Vivo Evidence for Lysosome Depletion and Impaired Autophagic Clearance in Hereditary Spastic Paraplegia Type SPG11
Fig 5
Purkinje cells in Spatacsin knockout mice accumulate abnormal autolysosomes.
(A-B”) While the small autofluorescent particles (AF) in WT Purkinje cells (A”) are rarely associated with the lysosomal membrane protein Lamp1, autofluorescent structures in KO Purkinje cells stain for Lamp1 at their periphery (B”). (C-D”) The autofluorescent material in KO Purkinje cells contains p62 (D”) suggesting that the vesicular structures positive for both Lamp1 and p62 represent autolysosomes. Purkinje cell somata are marked by a dashed line. Age at sampling: 16 months. AF: autofluorescence; Scale bars: 10 μm.