title: Plasma oligomeric alpha-synuclein is associated with glucocerebrosidase activity in Gaucher disease.
abstract: BACKGROUND
  The link between Parkinson's disease (PD) and Gaucher disease (GD), the most common lysosomal storage disease associated with loss of glucocerebrosidase (GBA) activity, can be explained by abnormal accumulation of oligomeric alpha-synuclein (α-Syn) species resulting from mutations in the GBA gene. However, in GD, the relationship between GBA activity and α-Syn accumulation in biological fluids has not been investigated.
  
  
  METHODS
  We analyzed plasma oligomeric α-Syn levels, leucocyte GBA activity, and plasma chitotriosidase activity in 21 patients with GD.
  
  
  RESULTS
  Negative correlation between plasma oligomeric α-Syn levels, and leucocyte GBA activity was observed in patients with GD (R(2)  = 0.487; P < 0.001).
  
  
  CONCLUSIONS
  The decrease in GBA activity may influence α-Syn oligomerization, explaining the high risk of PD development in GD patients. © 2015 International Parkinson and Movement Disorder Society.
authors.affiliation: First Pavlov's State Medical University of Saint-Petersburg, St. Petersburg, Russia.
authors.forename: Evgenii
authors.initials: E
authors.lastname: Nuzhnyi
authors.affiliation: First Pavlov's State Medical University of Saint-Petersburg, St. Petersburg, Russia.|Petersburg Nuclear Physics Institute, St. Petersburg, Russia.|St. Petersburg Academic University-Nanothecnology Research and Education Centre, RAS, St. Petersburg, Russia.
authors.forename: Anton
authors.initials: A
authors.lastname: Emelyanov
authors.affiliation: Medical-genetics Scientific Center, Moscow, Russia.
authors.forename: Tatyana
authors.initials: T
authors.lastname: Boukina
authors.affiliation: First Pavlov's State Medical University of Saint-Petersburg, St. Petersburg, Russia.|Petersburg Nuclear Physics Institute, St. Petersburg, Russia.
authors.forename: Tatiana
authors.initials: T
authors.lastname: Usenko
authors.affiliation: First Pavlov's State Medical University of Saint-Petersburg, St. Petersburg, Russia.
authors.forename: Andrey
authors.initials: A
authors.lastname: Yakimovskii
authors.affiliation: Medical-genetics Scientific Center, Moscow, Russia.
authors.forename: Ekaterina
authors.initials: E
authors.lastname: Zakharova
authors.affiliation: First Pavlov's State Medical University of Saint-Petersburg, St. Petersburg, Russia.|Petersburg Nuclear Physics Institute, St. Petersburg, Russia.|St. Petersburg Academic University-Nanothecnology Research and Education Centre, RAS, St. Petersburg, Russia.
authors.forename: Sofya
authors.initials: S
authors.lastname: Pchelina
mesh_terms: D000368:Aged; D002648:Child; D002675:Child, Preschool; D005260:Female; D005776:Gaucher Disease / Q000097:blood*; D005962:Glucosylceramidase / Q000097:blood*; D006801:Humans; D007223:Infant; D008297:Male; D051844:alpha-Synuclein / Q000097:blood*
chemical_list: D051844:alpha-Synuclein; D005962:Glucosylceramidase
keywords: Gaucher disease; Parkinson's disease; alpha-synuclein; glucocerebrosidase; oligomerization