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. Author manuscript; available in PMC: 2021 Apr 1.
Published in final edited form as: Amyotroph Lateral Scler Frontotemporal Degener. 2020 Nov;21(SUP1):35–46. doi: 10.1080/21678421.2020.1837175

Figure 2.

Figure 2

GFP-labeling of CSMN in Alsin KO mice reveals significant cellular problems in the absence of Alsin function. (A) AlsinKO-UeGFP mice are generated by crossing the UCHL1-eGFP and the AlsinKO mice and in these mice the CSMN are genetically labeled with eGFP that is stable and long-lasting (57). (B–C) The coupled immuno-electron microscopy analyses, reveal that the diseased CSMN cannot maintain the cytoarchitectural integrity of their apical dendrites (B), have massive mitochondrial defects with collapsed mitochondria that are cleared by mitophagy (C). (D) The Golgi apparatus is enlarged and the vesicles may not fuse properly. These cellular defects are not detected in healthy CSMNs.