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

L. Parnetti, C. Balducci, L. Pierguidi, C. De Carlo, M. Peducci, C. D'Amore, C. Padiglioni, S. Mastrocola, E. Persichetti, S. Paciotti, G. Bellomo, N. Tambasco, A. Rossi, T. Beccari, P. Calabresi

Research output: Contribution to journalArticle

Abstract

The autophagy-lysosomal degradation pathway plays a role in the onset and progression of neurodegenerative diseases. Clinical and genetic studies indicate that mutations of β-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 β-glucocerebrosidase, in cerebrospinal fluid of PD patients. We have thus measured the activity of these enzymes - α-mannosidase (EC 3.2.1.24), β-mannosidase (EC 3.2.1.25), β-glucocerebrosidase (EC 3.2.1.45), β-galactosidase (EC 3.2.1.23) and β-hexosaminidase (EC 3.2.1.52) - 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, β-glucocerebrosidase activity was selectively reduced in DLB, further suggesting that this enzyme might specifically be impaired in synucleinopathies.

Original languageEnglish
Pages (from-to)484-486
Number of pages3
JournalNeurobiology of Disease
Volume34
Issue number3
DOIs
Publication statusPublished - Jun 2009

Keywords

  • α-mannosidase
  • β-glucocerebrosidase
  • Alzheimer's Disease
  • Cerebrospinal fluid
  • Dementia with Lewy Bodies
  • Fronto-Temporal Dementia
  • Lysosomal enzymes
  • Lysosomal hydrolases
  • Synucleinopathies

ASJC Scopus subject areas

  • Neurology

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  • Cite this

    Parnetti, L., Balducci, C., Pierguidi, L., De Carlo, C., Peducci, M., D'Amore, C., Padiglioni, C., Mastrocola, S., Persichetti, E., Paciotti, S., Bellomo, G., Tambasco, N., Rossi, A., Beccari, T., & Calabresi, P. (2009). Cerebrospinal fluid β-glucocerebrosidase activity is reduced in Dementia with Lewy Bodies. Neurobiology of Disease, 34(3), 484-486. https://doi.org/10.1016/j.nbd.2009.03.002