Huntingtin fragments form aggresome-like inclusion bodies in mammalian cells

The formation of large perinuclear inclusion bodies containing protein aggregates was first described in HeLa cells. Woijcik et al. (1) have shown that treatment of HeLa cells with the proteasome inhibitor PSI [N-benzylo-xycarbonal-Ile-Glu(O-t-butyl)-Ala-leucinal] results in the accumulation of electron-dense material in the vicinity of the Golgi apparatus. These structures were termed proteolysis centers, because ubiquitin as well as components of the 26S proteasome are enriched in inclusion bodies, whereas carbohydrates, lipids, or nucleic acids are not present.