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Alpha galactosidase A activity in Parkinson's disease
R N Alcalay1, P Wolf2, O A Levy1
1Department of Neurology, College of Physicians and Surgeons, Columbia University Medical Center, New York, NY, USA; Taub Institute for Research on Alzheimer's Disease and the Aging Brain, College of Physicians and Surgeons, Columbia University Medical Center, New York, NY, USA.
Neurobiology of Disease
|January 26, 2018
Summary
Reduced alpha galactosidase A activity is linked to Parkinson's disease (PD) risk, suggesting a role beyond glucocerebrosidase. This study investigated lysosomal enzyme activity in PD patients, finding lower alpha galactosidase A levels.
Area of Science:
- Neuroscience
- Biochemistry
- Genetics
Background:
- Glucocerebrosidase (GCase) deficiency is linked to Gaucher disease and has been observed in Parkinson's Disease (PD).
- It remains unclear if other lysosomal enzyme activities are also reduced in PD.
- Investigating additional lysosomal enzymes can elucidate PD pathogenesis and potential biomarkers.
Purpose of the Study:
- To determine if reduced lysosomal enzyme activity in PD is specific to GCase or a broader phenomenon.
- To compare enzymatic activities of GCase, acid sphingomyelinase, alpha galactosidase A, acid alpha-glucosidase, and galactosylceramidase in PD patients versus controls.
- To explore the independent role of alpha galactosidase A in PD, beyond GCase.
Main Methods:
- Enzymatic activities were measured in dried blood spots from 648 PD patients and 317 controls.
- Glucocerebrosidase (GBA) and LRRK2 G2019S mutation status were determined via full sequencing.
- Statistical analyses, including t-tests and regression models adjusted for covariates, were used to compare enzyme activities.
Main Results:
- Alpha galactosidase A activity was significantly lower in PD cases compared to controls, even after excluding GBA/LRRK2 carriers and young-onset PD.
- This reduction in alpha galactosidase A activity was also observed in women who were non-carriers of GBA and LRRK2 mutations.
- Activities of acid sphingomyelinase, acid alpha-glucosidase, and galactosylceramidase did not differ significantly between PD and controls.
Conclusions:
- Reduced alpha galactosidase A activity is associated with Parkinson's Disease, suggesting a potential independent role.
- The findings indicate that lysosomal enzyme alterations in PD may extend beyond GCase.
- Alpha galactosidase A warrants further investigation as a potential biomarker or therapeutic target in Parkinson's Disease.
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