IKK phosphorylates Huntingtin and targets it for degradation by the proteasome and lysosome

J Cell Biol. 2009 Dec 28;187(7):1083-99. doi: 10.1083/jcb.200909067. Epub 2009 Dec 21.

Abstract

Expansion of the polyglutamine repeat within the protein Huntingtin (Htt) causes Huntington's disease, a neurodegenerative disease associated with aging and the accumulation of mutant Htt in diseased neurons. Understanding the mechanisms that influence Htt cellular degradation may target treatments designed to activate mutant Htt clearance pathways. We find that Htt is phosphorylated by the inflammatory kinase IKK, enhancing its normal clearance by the proteasome and lysosome. Phosphorylation of Htt regulates additional post-translational modifications, including Htt ubiquitination, SUMOylation, and acetylation, and increases Htt nuclear localization, cleavage, and clearance mediated by lysosomal-associated membrane protein 2A and Hsc70. We propose that IKK activates mutant Htt clearance until an age-related loss of proteasome/lysosome function promotes accumulation of toxic post-translationally modified mutant Htt. Thus, IKK activation may modulate mutant Htt neurotoxicity depending on the cell's ability to degrade the modified species.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Brain / metabolism
  • Cell Line
  • Humans
  • Huntingtin Protein
  • I-kappa B Kinase / physiology*
  • Lysosomes / metabolism*
  • Mice
  • Nerve Tissue Proteins / analysis
  • Nerve Tissue Proteins / chemistry
  • Nerve Tissue Proteins / metabolism*
  • Nuclear Proteins / analysis
  • Nuclear Proteins / chemistry
  • Nuclear Proteins / metabolism*
  • Phosphorylation
  • Proteasome Endopeptidase Complex / metabolism*
  • Protein Structure, Tertiary
  • Rats
  • Rats, Sprague-Dawley
  • Solubility
  • Ubiquitination

Substances

  • Htt protein, rat
  • Huntingtin Protein
  • Nerve Tissue Proteins
  • Nuclear Proteins
  • I-kappa B Kinase
  • Proteasome Endopeptidase Complex