Physiological and pathological role of alpha-synuclein in Parkinson's disease through iron mediated oxidative stress; the role of a putative iron-responsive element

Int J Mol Sci. 2009 Mar;10(3):1226-60. doi: 10.3390/ijms10031226. Epub 2009 Mar 17.

Abstract

Parkinson's disease (PD) is the second most common progressive neurodegenerative disorder after Alzheimer's disease (AD) and represents a large health burden to society. Genetic and oxidative risk factors have been proposed as possible causes, but their relative contribution remains unclear. Dysfunction of alpha-synuclein (alpha-syn) has been associated with PD due to its increased presence, together with iron, in Lewy bodies. Brain oxidative damage caused by iron may be partly mediated by alpha-syn oligomerization during PD pathology. Also, alpha-syn gene dosage can cause familial PD and inhibition of its gene expression by blocking translation via a newly identified Iron Responsive Element-like RNA sequence in its 5'-untranslated region may provide a new PD drug target.

Keywords: 5-UTR: 5’-untranslated region; 6-OHDA: 6-hydroxydopamine; AD: Alzheimer’s disease; CNS: central nervous system; DA: dopamine; DAT: dopamine transporter; DLB: dementia with Lewy Bodies; ER: endoplasmatic reticulum; GCIs: glial cytoplasmic inclusions; GSH: reduced gluthatione; IRE: iron responsive element; IRPs: interacting binding proteins; LBs: Lewy bodies; LNs: Lewy neurites; MPTP: 1-methyl 4-phenyl 1, 2, 3, 6 tetrapyridine; NAC: non-amyloidogenic component; PD: Parkinson’s disease; PLD2: phospholipase D2; PM: plasmatic membrane; ROS: reactive oxygen species; TH: tyrosine hydroxylase; TfR: transferrin receptor; aa: amino acid(s); nt: nucleotide(s); wt: wild-type; α-syn: alpha-synuclein.

Publication types

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

MeSH terms

  • Animals
  • Ferric Compounds / chemistry
  • Ferric Compounds / toxicity*
  • Humans
  • Levodopa / therapeutic use
  • Lewy Bodies / metabolism
  • Neuroprotective Agents / therapeutic use
  • Oxidative Stress / drug effects*
  • Parkinson Disease / drug therapy
  • Parkinson Disease / metabolism
  • Parkinson Disease / physiopathology*
  • Protein Binding
  • Protein Processing, Post-Translational
  • RNA / metabolism
  • alpha-Synuclein / chemistry
  • alpha-Synuclein / genetics
  • alpha-Synuclein / metabolism*

Substances

  • Ferric Compounds
  • Neuroprotective Agents
  • alpha-Synuclein
  • Levodopa
  • RNA