The oxysterol 27-hydroxycholesterol regulates α-synuclein and tyrosine hydroxylase expression levels in human neuroblastoma cells through modulation of liver X receptors and estrogen receptors--relevance to Parkinson's disease

J Neurochem. 2011 Dec;119(5):1119-36. doi: 10.1111/j.1471-4159.2011.07497.x. Epub 2011 Oct 24.

Abstract

Loss of dopaminergic neurons and α-synuclein accumulation are the two major pathological hallmarks of Parkinson's disease. Currently, the mechanisms governing depletion of dopamine content and α-synuclein accumulation are not well understood. We showed that the oxysterol 27-hydroxycholesterol (27-OHC) reduces the expression of tyrosine hydroxylase (TH), the rate-limiting enzyme in dopamine synthesis, and increases α-synuclein levels in SH-SY5Y cells. However, the cellular mechanisms involved in 27-OHC effects were not elucidated. In this study, we demonstrate that 27-OHC regulates TH and α-synuclein expression levels through the estrogen receptors (ER) and liver X receptors (LXR). We specifically show that inhibition of ERβ mediates 27-OHC-induced decrease in TH expression, an effect reversed by the ER agonist estradiol. We also show that 27-OHC and the LXR agonist GW3965 increase α-synuclein while the LXR antagonist 5α-6α-epoxycholesterol-3-sulfate significantly attenuated the 27-OHC-induced increase in α-synuclein expression. We further demonstrate that LXRβ positively regulates α-synuclein expression and 27-OHC increases LXRβ-mediated α-synuclein transcription. Our results demonstrate the involvement of two distinct pathways that are involved in the 27-OHC regulation of TH and α-synuclein levels. Concomitant activation of ERβ and inhibition of LXRβ prevent 27-OHC effects and may therefore reduce the progression of Parkinson's disease by precluding TH reduction and α-synuclein accumulation.

Publication types

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

MeSH terms

  • Cell Line, Tumor
  • Disease Progression
  • Down-Regulation / drug effects
  • Down-Regulation / physiology
  • Estrogen Receptor alpha / agonists
  • Estrogen Receptor alpha / antagonists & inhibitors
  • Estrogen Receptor alpha / biosynthesis*
  • Estrogen Receptor beta / agonists
  • Estrogen Receptor beta / antagonists & inhibitors
  • Estrogen Receptor beta / biosynthesis*
  • Humans
  • Hydroxycholesterols / pharmacology*
  • Liver X Receptors
  • Neuroblastoma / enzymology
  • Neuroblastoma / metabolism*
  • Neuroblastoma / pathology
  • Orphan Nuclear Receptors / agonists
  • Orphan Nuclear Receptors / antagonists & inhibitors
  • Orphan Nuclear Receptors / biosynthesis*
  • Parkinson Disease / enzymology
  • Parkinson Disease / metabolism
  • Parkinson Disease / prevention & control*
  • Signal Transduction / genetics
  • Signal Transduction / physiology
  • Tyrosine 3-Monooxygenase / antagonists & inhibitors*
  • Tyrosine 3-Monooxygenase / biosynthesis
  • Tyrosine 3-Monooxygenase / genetics
  • Up-Regulation / drug effects
  • Up-Regulation / physiology
  • alpha-Synuclein / biosynthesis
  • alpha-Synuclein / genetics*

Substances

  • Estrogen Receptor alpha
  • Estrogen Receptor beta
  • Hydroxycholesterols
  • Liver X Receptors
  • Orphan Nuclear Receptors
  • SNCA protein, human
  • alpha-Synuclein
  • 27-hydroxycholesterol
  • Tyrosine 3-Monooxygenase