(A) Proteasome 26S and 20S chymotrypsin-like activity. *p<0,05 (B) Densitometry analysis of total ubiquitinated protein content and representative WB. (C) Ubiquitination influence on proteasome activity in LOPD patients: 26S chymotrypsin-like activity do not correlated with ubiquitinated protein content. N=13, Pearson r=0.2840, p=0.3470, r2=0.08066; 20S chymotrypsin-like activity has a significant positive correlation with ubiquitinated protein content. N=13, Pearson r=0.6486, *P<0,05, r2=0,4207.