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. 2019 May 23;9(2):315–326. doi: 10.3233/JPD-191590

Fig.1.

Fig.1

Inducing α-syn pathology in En1+/– mice. Engrailed1 expression is vital for CNS development and maintenance, particularly for nigrostriatal projections. A) En1+/– mice feature a LacZ insertion cassette in one allele and feature B) progressive “dying-back” nigrostriatal degeneration, dysfunctional mechanisms in the autophagy-lysosomal pathway, and pathologic neuroinflammatory activation. En1+/– mice also feature mitochondrial respiratory chain perturbations and motor dysfunction associated with PD clinical pathology [7, 9]. C) Human pre-formed α-syn fibrils were sonicated, validated by TEM, and injected into the dorsal striatum. D) PFF injection causes robust phosphorylated-serine129 α-syn (pS129) deposition at the site of injection. E) Both WT and En1+/– animals were unilaterally injected at one month of age and sacrificed for post-mortem histology eight weeks following PFF injection.