Proteasomes cleave at multiple sites within polyglutamine tracts: activation by PA28gamma(K188E)

J Biol Chem. 2008 May 9;283(19):12919-25. doi: 10.1074/jbc.M709347200. Epub 2008 Mar 13.

Abstract

Eukaryotic proteasomes have been reported to cleave only once within polyglutamine tracts and then only after the N-terminal glutamine (Venkatraman, P., Wetzel, R., Tanaka, M., Nukina, N., and Goldberg, A. L. (2004) Mol. Cell 14, 95-104). We have obtained results that directly conflict with that report. In the presence of the proteasome activator PA28gamma(K188E) human red cell proteasomes progressively degraded fluorescein-GGQ(10)RR or fluorescein-HPHQ(10)RR into small fragments as shown by size exclusion chromatography and mass spectrometry. MALDI-TOF mass spectrometry revealed that proteolytic products arose from cleavage after every glutamine in fluorescein-HPHQ(10)RR, and mass accuracy rules out deamidation of glutamine to glutamic acid as an explanation for peptide degradation. Moreover, degradation cannot be attributed to a contaminating protease because peptide hydrolysis was completely blocked by the proteasome-specific inhibitors, lactacystin and epoxomicin. We conclude that proteasomes cleave repetitively anywhere within a stretch of ten glutamine residues. Thus our results cast doubt on the idea that mammalian proteasomes cannot degrade glutamine-expanded regions within pathogenic polyQ-expanded proteins, such as Huntingtin.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Enzyme Activation
  • Humans
  • Ions / chemistry
  • Mass Spectrometry
  • Mutation / genetics
  • Peptides / chemistry
  • Peptides / metabolism*
  • Proteasome Endopeptidase Complex / genetics
  • Proteasome Endopeptidase Complex / metabolism*

Substances

  • Ions
  • Peptides
  • polyglutamine
  • Proteasome Endopeptidase Complex