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

41 Citations (Scopus)

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

Fingerprint

Glucosylceramidase
Lewy Body Disease
Cerebrospinal Fluid
Mannosidases
Parkinson Disease
Dementia
Galactosidases
alpha-Mannosidase
Hexosaminidases
Autophagy
Hydrolases
Enzymes
Neurodegenerative Diseases
Alzheimer Disease
Mutation

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

Cite this

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

In: Neurobiology of Disease, Vol. 34, No. 3, 06.2009, p. 484-486.

Research output: Contribution to journalArticle

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, vol. 34, no. 3, pp. 484-486. https://doi.org/10.1016/j.nbd.2009.03.002
Parnetti L, Balducci C, Pierguidi L, De Carlo C, Peducci M, D'Amore C et al. Cerebrospinal fluid β-glucocerebrosidase activity is reduced in Dementia with Lewy Bodies. Neurobiology of Disease. 2009 Jun;34(3):484-486. https://doi.org/10.1016/j.nbd.2009.03.002
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. / Cerebrospinal fluid β-glucocerebrosidase activity is reduced in Dementia with Lewy Bodies. In: Neurobiology of Disease. 2009 ; Vol. 34, No. 3. pp. 484-486.
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