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. Author manuscript; available in PMC: 2019 Sep 1.
Published in final edited form as: Acta Neuropathol. 2018 May 3;136(3):425–443. doi: 10.1007/s00401-018-1852-9

Figure 7. SETX ALS4 mice exhibit nuclear membrane abnormalities.

Figure 7

(a) We immunostained L5 lumbar spinal cord sections from 11.5 month-old mice of the indicated genotypes for Ran, and counterstained with DAPI. The nuclear membrane was usually smooth without invagination in motor neurons from Non-Tg mice, but displayed frequent irregularities in SETX-L389S+/− mice, such as this invagination (yellow arrow). Scale bar = 20 μm

(b) We immunostained L5 lumbar spinal cord sections from 11.5 month-old mice of the indicated genotypes for RanGAP1, and counterstained with DAPI. The nuclear membrane outline was typically round and regular without invagination in motor neurons from Non-Tg mice, but displayed frequent abnormalities in SETX-L389S+/− mice, such as coffee bean-like invaginations (yellow arrows). Scale bar = 20 μm

(c) Quantification of nuclear membrane abnormalities in motor neurons from Non-Tg and SETX-L389S+/− mice in (a & b). n = 3 mice/genotype, 50 – 80 motor neurons/individual mouse. *P < .05, **P < .01, t-test.