The SQSTM1 L341V Variant Associated With Sporadic ALS Promotes the Accumulation of Enlarged Ubiquitin-Positive SQSTM1 Bodies.

In the L341V group, there was a nonsignificant trend toward a decrease in number of photoconverted SQSTM1‐bodies from the “after” to the first period (Figure 4E and Figure S7D ).

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In the L341V group, the size of unconverted SQSTM1‐bodies showed a trend toward an increase from immediately before photoconversion (“before”) up to 3 h after photoconversion (“period 2/2nd”), although these differences were not statistically significant (Figure 2B and Figure S3A ).

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In contrast, Dendra2 visualization revealed a decreasing trend in the number of SQSTM1‐bodies present at the time of photoconversion, suggesting the involvement of proteolysis. 2.2 The LC3 ‐Affinity‐Defective SQSTM1 L341V Variant Forms Larger SQSTM1 ‐Bodies Than Either the Wild‐Type or the Artificial LIR3A Mutant Under Autophagy‐Activating Conditions To examine whether the differences in SQSTM1‐body dynamics observed under normal culture conditions were influenced by autophagy, we analyzed their behavior in cells subjected to autophagy‐activating conditions via serum starvation, followed by photoconversion (Figure 3A and Figure S4 ).

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