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The SPID-GBA study

Letizia Straniero, Rosanna Asselta, Salvatore Bonvegna, Valeria Rimoldi, Giada Melistaccio, Giulia Soldà, Massimo Aureli, Matteo Giovanni Della Porta, Ugo Lucca, Alessio Di Fonzo, Anna Zecchinelli, Gianni Pezzoli, Roberto Cilia, Stefano Duga

2020Neurology Genetics65 citationsDOIOpen Access PDF

Abstract

OBJECTIVE: variants, we analyzed a large cohort of 4,923 Italian unrelated patients with primary degenerative parkinsonism (including 3,832 PD) enrolled in a single tertiary care center and 7,757 ethnically matched controls. METHODS: The p.E326K, p.T369M, p.N370S, and p.L444P variants were screened using an allele-specific multiplexed PCR approach. All statistical procedures were performed using R or Plink v1.07. RESULTS: ), which, on the basis of the impact on glucocerebrosidase activity, would be expected to be mild. The 1.5-2:1 male sex bias described in sporadic PD was lost in p.T369M carriers. We also showed that PD penetrance for p.L444P could reach the 15% at age 75 years. CONCLUSIONS: .

Topics & Concepts

Odds ratioDementiaDementia with Lewy bodiesGlucocerebrosidasePenetranceParkinsonismMedicineInternal medicineConfidence intervalIncidence (geometry)AlleleGastroenterologyParkinson's diseaseDiseaseGeneticsBiologyGeneOpticsPhysicsPhenotypeLysosomal Storage Disorders ResearchParkinson's Disease Mechanisms and TreatmentsNeurological diseases and metabolism
The SPID-GBA study | Litcius