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. 2019 Jan 15;142(2):276–294. doi: 10.1093/brain/awy330

Figure 5.

Figure 5

SYNCRIP protects human SMA motor neurons from degeneration by increasing NRXN2 and SMN levels. (A) Representative images of SMA motor neurons after SYNCRIP transfection (Hb9::eGFP, green; SYNCRIP, red). Nuclei are labelled with DAPI (blue). (B) q-PCR analysis showed a significant increase in the expression of SYNCRIP, SMN, and NRXN2 transcripts in SMA-motor neurons compared to null SMA-motor neurons. ***P < 0.001, student t-test. Data are presented as mean ± SEM from three independent experiments. (C) Western blot representative image of SYNCRIP, SMN, and NRXN2 in SMA motor neurons after SYNCRIP overexpression compared to SMA-null cells. Western blot analysis revealed an increased level of SYNCRIP (*P < 0.05), SMN (*P < 0.05), and NRXN2 (*P < 0.05) in SMA motor neurons after SYNCRIP overexpression compared to SMA-null cells. Data are presented as mean ± SEM of three independent experiments. (D) Representative images of SMA motor neurons (Hb9::eGFP, green; ChAT, red) with and without SYNCRIP transfection. Nuclei are labelled with DAPI (blue). (E) The number of SYNCRIP SMA-motor neurons in long-term culture was significantly increased compared to SMA motor neurons null. SYNCRIP overexpression was protective to motor neurons. ***P < 0.0001, Student t-test. Data are presented as mean ± SEM of five independent experiments. (F) At 8 weeks, SMA motor neurons overexpressing SYNCRIP showed longer axons than SMA motor neurons null. P < 0.001, Kolmogorov-Smirnov test. Scale bar = 75 µm.