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Expanded (pathogenic) AT3 is also activated by ubiquitination. (A) Recombinant, expanded AT3 (AT3(Q80)) was ubiquitinated in vitro, then incubated with the indicated Ub chains. AT3(Q80)-Ub shows greater enhancement of activity towards K63-Ub6 chains. (B) Normal and expanded AT3 show similar enhancement in activity when ubiquitinated. Results in (A) and (B) are each representative of three independent experiments. Anti-AT3 blots show GST–AT3 species used in reactions.
