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. Author manuscript; available in PMC: 2019 Dec 1.
Published in final edited form as: J Mol Med (Berl). 2018 Oct 20;96(12):1359–1373. doi: 10.1007/s00109-018-1703-0

Fig. 2. PGRN deficiency causes a TSD-like phenotype.

Fig. 2

(a) IHC staining of GM2 in paraffinembedded brain tissue slides from aged (one-year old) wild-type and aged PGRN knockout mice. The HexA substrate, GM2, was aggregated in aged PGRN KO mice. (b) Immunofluorescence staining of GM2 in paraffin-embedded brain tissue slides from aged wild-type and PGRN knockout mice. The fluorescence intensity was greatly increased in aged PGRN KO mice relative to aged wild-type mice. (c) Confocal staining of GM2 in frozen brain sections from aged WT and PGRN KO mice. (Red: GM2 Blue: DAPI). (d) Transmission electron microscope analysis of lung tissue from PGRN knockout mice challenged with OVA (upper panel) and fibroblasts from TSD patients (lower panel). The appearance of lysosomes changed from regular round shapes to tubular-like shapes (red arrow) and zebra bodies, multilayer membranous structures (blue arrow), appeared in PGRN KO macrophages. Zebra bodies in TSD fibroblasts served as a positive control (lower panel). (e) IHC staining of HexA and HexB in paraffinembedded lung slides from wild type and PGRN KO mice challenged by OVA. HexA was accumulated while HexB was evenly distributed in macrophages. Aggregation of HexA in macrophages was marked by a red arrow. (f) IHC staining of GM2 in brain tissue from PGRN knockout mice treated with or without OVA. GM2 was dramatically aggregated in PGRN KO mice. (g) Immunofluorescence staining of GM2 in brain tissue from PGRN knockout mice treated with or without OVA. Fluorescence intensity was markedly increased in PGRN KO mice. (h) Confocal staining of GM2 in frozen brain sections from PGRN KO mice, with or without OVA challenge (Red: GM2, Blue: DAPI).