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. Author manuscript; available in PMC: 2018 May 4.
Published in final edited form as: Acta Neuropathol. 2017 Dec 1;135(3):459–474. doi: 10.1007/s00401-017-1793-8

Figure 5. C9orf72 protein is widely distributed in cytoplasm of neurons and glia throughout the CNS, but is reduced in both disease-related and disease-unrelated regions.

Figure 5

(a–b) IF staining show C9orf72 protein is present in neurons of granular, molecular and Purkinje layer of cerebellum of wildtype mice, which is absent in C9 knockout mice.

(c–d) IHC staining of C9or72 of Betz cells in layer 5 of motor cortex (c, insert) and motor neurons of anterior horn of spinal cord (d, insert) of C9 ALS case show diffuse cytoplasmic staining with C9orf72 antibody.

(e–l) C9orf72 IHC staining shows no significant differences in diffuse cytoplasmic staining in Betz cells of motor cortex (layer 5), big neurons of frontal cortex (layer 5), Purkinje cells of cerebellum, and spinal motor neurons of anterior horn of spinal cord between C9 ALS and SALS.

(m–p) Western blotting shows that C9orf72 protein expression is decreased in frontal cortex (m–n) and in occipital cortex (o–p) of C9 ALS compared to control and SALS cases.