title: Decreased activities of lysosomal acid alpha-D-galactosidase A in the leukocytes of sporadic Parkinson's disease.
abstract: Parkinson's disease (PD) is a progressive neurodegenerative disease that affects aged people. Although a number of genes have been linked to familial PD, the genetic causes of sporadic PD that accounts for 90% of all PD cases remain unclear. Accumulating evidence has demonstrated that alpha-synuclein aggregation is essential to the pathogenesis of PD. Recent studies suggest that autophagic-lysosomal system play major roles in the process of alpha-synuclein aggregation. We hypothesized that lysosomal acid hydrolases may be involved in the alpha-synuclein degradation and aggregation. In this study, we examined the activities of 11 lysosomal acid hydrolases in peripheral blood leukocytes of 38 sporadic PD patients and 258 age- and sex-matched healthy controls. The activities of alpha-D-galactosidase A were significantly decreased in sporadic PD patients, compared to age- and sex-matched controls. In contrast, no significant differences of the activities of other 10 lysosomal acid hydrolases was observed. This initial study suggests that decreased activities of lysosomal alpha-D-galactosidase A in the central nervous system may be involved in the degradation and aggregation of alpha-synuclein protein and contribute to the pathogenesis of sporadic PD as a risk factor.
authors.affiliation: Department of Medicine, Jining Medical College, Jining, Shandong 272029, PR China.
authors.forename: Guanghua
authors.initials: G
authors.lastname: Wu
authors.forename: Bo
authors.initials: B
authors.lastname: Yan
authors.forename: Xuenan
authors.initials: X
authors.lastname: Wang
authors.forename: Xungang
authors.initials: X
authors.lastname: Feng
authors.forename: Aimei
authors.initials: A
authors.lastname: Zhang
authors.forename: Xintan
authors.initials: X
authors.lastname: Xu
authors.forename: Haixin
authors.initials: H
authors.lastname: Dong
mesh_terms: D000328:Adult; D000367:Age Factors; D000368:Aged; D000704:Analysis of Variance; D005260:Female; D006801:Humans; D007962:Leukocytes / Q000201:enzymology*; D008297:Male; D008875:Middle Aged; D010300:Parkinson Disease / Q000473:pathology*; D000519:alpha-Galactosidase / Q000378:metabolism*; D051844:alpha-Synuclein / Q000378:metabolism
chemical_list: D051844:alpha-Synuclein; D000519:alpha-Galactosidase