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. 2006 Apr 24;103(18):7142–7147. doi: 10.1073/pnas.0602046103

Fig. 3.

Fig. 3.

Detergent-insoluble form of both mutant and WT SOD1 in spinal cord of affected transgenic mice. (a) SOD1 in the supernatant fraction detected with hs-SOD1 (Top) and f-SOD1 (Middle) antibodies in different mouse lines. NT, nontransgenic mice. β-Actin was used as a control (Bottom). Mouse endogenous SOD1 is indicated by an arrow. Human SOD1 is used as a standard (stSOD1). (b) SOD1 in the pellet fraction detected with f-SOD1 antibody. A certain amount of hwtSOD1 could be detected in hwtSOD1 transgenic mice (Upper, arrowhead), but this hwtSOD1 is not detergent-insoluble (Lower). The detergent-insoluble form of SOD1 was detected in the affected SOD1A4V/hwtSOD1 double-transgenic mice but not in the unaffected SOD1A4V single-transgenic mice. The truncated SOD1L126Z protein was detected only in the insoluble fraction and was present in a detergent-insoluble form (arrow). Different from the hwtSOD1 in the hwtSOD1 transgenic mice, the hwtSOD1 in the SOD1L126Z/hwtSOD1 double-transgenic mice is detergent-insoluble (arrowhead). Similar results were obtained by using the hs-SOD1 antibody.