PH domain leucine-rich repeat protein phosphatase 1 contributes to maintain the activation of the PI3K/Akt pro-survival pathway in Huntington's disease striatum

Cell Death Differ. 2010 Feb;17(2):324-35. doi: 10.1038/cdd.2009.127. Epub 2009 Sep 11.

Abstract

Dysregulation of gene expression is one of the mechanisms involved in the pathophysiology of Huntington's disease (HD). Here, we examined whether mutant huntingtin regulates the levels of PH domain leucine-rich repeat protein phosphatase 1 (PHLPP1), a phosphatase that specifically dephosphorylates Akt at Ser473. Our results show decreased PHLPP1 protein levels in knock-in models (Hdh(Q111/Q111) mouse striatum and STHdh(Q111/Q111) cells), in the striatum of N-terminal exon-1 mutant huntingtin transgenic mouse models (R6/1; R6/1 : BDNF + or - , R6/2 and Tet/HD94) and in the putamen of HD patients. Quantitative PCR analysis revealed a reduction in PHLPP1 mRNA levels in the striatum of R6/1 compared with wild-type mice. Coincident with reduced PHLPP1 protein levels, we observed increased phosphorylated Akt (Ser473) levels specifically in the striatum. The analysis of the conditional mouse model Tet/HD94 disclosed that after mutant huntingtin shutdown PHLPP1 levels returned to wild-type levels whereas phospho-Akt levels were partially reduced. In conclusion, our results show that mutant huntingtin downregulates PHLPP1 expression. In the striatum, these reduced levels of PHLPP1 can contribute to maintain high levels of activated Akt that may delay cell death and allow the recovery of neuronal viability after mutant huntingtin silencing.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Animals
  • Cell Death / physiology
  • Cell Line, Transformed
  • Cell Nucleus / metabolism
  • Corpus Striatum / enzymology*
  • Corpus Striatum / pathology
  • Cytosol / metabolism
  • Disease Models, Animal
  • Exons / genetics
  • Female
  • Gene Knock-In Techniques
  • Humans
  • Huntington Disease / enzymology*
  • Huntington Disease / genetics
  • Huntington Disease / pathology
  • Male
  • Mice
  • Mice, Transgenic
  • Middle Aged
  • Neurotoxins / metabolism
  • Nuclear Proteins / chemistry
  • Nuclear Proteins / genetics
  • Nuclear Proteins / metabolism*
  • Phosphatidylinositol 3-Kinases / metabolism*
  • Phosphoprotein Phosphatases / chemistry
  • Phosphoprotein Phosphatases / genetics
  • Phosphoprotein Phosphatases / metabolism*
  • Phosphorylation / physiology
  • Protein Structure, Tertiary
  • Proto-Oncogene Proteins c-akt / metabolism*

Substances

  • Neurotoxins
  • Nuclear Proteins
  • Phosphatidylinositol 3-Kinases
  • Proto-Oncogene Proteins c-akt
  • PHLPP1 protein, human
  • Phosphoprotein Phosphatases