Cerebrospinal fluid beta-glucocerebrosidase activity is reduced in Dementia with Lewy Bodies

Neurobiol Dis. 2009 Jun;34(3):484-6. doi: 10.1016/j.nbd.2009.03.002. Epub 2009 Mar 20.


The autophagy-lysosomal degradation pathway plays a role in the onset and progression of neurodegenerative diseases. Clinical and genetic studies indicate that mutations of beta-glucocerebrosidase represent genetic risk factors for synucleinopathies, including Parkinson's Disease (PD) and Dementia with Lewy Bodies (DLB). We recently found a decreased activity of lysosomal hydrolases, namely beta-glucocerebrosidase, in cerebrospinal fluid of PD patients. We have thus measured the activity of these enzymes - alpha-mannosidase (EC, beta-mannosidase (EC, beta-glucocerebrosidase (EC, beta-galactosidase (EC and beta-hexosaminidase (EC - in cerebrospinal fluid of patients suffering from DLB, Alzheimer's Disease (AD), Fronto-Temporal Dementia (FTD) and controls. Alpha-mannosidase activity showed a marked decrease across all the pathological groups as compared to controls. Conversely, beta-glucocerebrosidase activity was selectively reduced in DLB, further suggesting that this enzyme might specifically be impaired in synucleinopathies.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Alzheimer Disease / cerebrospinal fluid
  • Alzheimer Disease / enzymology
  • Dementia / cerebrospinal fluid
  • Dementia / enzymology
  • Female
  • Glucosylceramidase / cerebrospinal fluid*
  • Humans
  • Lewy Body Disease / cerebrospinal fluid*
  • Lewy Body Disease / enzymology*
  • Male
  • Middle Aged
  • alpha-Mannosidase / cerebrospinal fluid
  • beta-Galactosidase / cerebrospinal fluid
  • beta-Mannosidase / cerebrospinal fluid
  • beta-N-Acetylhexosaminidases / cerebrospinal fluid


  • beta-Galactosidase
  • alpha-Mannosidase
  • beta-Mannosidase
  • Glucosylceramidase
  • beta-N-Acetylhexosaminidases