Fig. 6.
Protein aggregation in CCS/G93A-SOD1 dual mice. (A and B) Western blots of spinal cord extracts from 24-day-old G93A-SOD1, CCS/G93A-SOD1 dual, and CCS/WT-SOD1 dual mice probed for CCS (A) and SOD1 (B). (C) Western blot showing CCS and SOD1 expression in spinal cord extracts from 21-day-old WT-SOD1 transgenic and CCS/ WT-SOD1 dual mice. (D) Western blots of spinal cord extracts from 24-day-old CCS/G93A-SOD1 dual and 8-month-old G93A-SOD1 mice probed for SOD1. (E and F) Immunohistochemistry of lumbar spinal cord sections from weak 8-month-old G93A-SOD1 (E) and weak 34-day-old CCS/G93A-SOD1 dual mice (F). Merged images stained for ubiquitin (red), NeuN (green), and DAPI (blue). Arrow shows skein-like inclusions; arrowheads show Lewy-like inclusions. Identical exposure times. (Scale bars: 20 μm.) (G) Western blot of extracts from spinal cord cultures derived from 8-day-old nontransgenic, G93A-SOD1, or CCS/G93A-SOD1 dual mice with (+) or without (−) lactacystin treatment compared with 8-month-old G93A-SOD1 mouse. Western blots probed for SOD1 (20 μg of protein per lane).