Glucocerebrosidase mutations are an important risk factor for Lewy body disorders.

Goker-Alpan, O; Giasson, B I; Eblan, M J; Nguyen, J; Hurtig, H I; Lee, V M-Y; Trojanowski, J Q; Sidransky, E · Neurology · 2006

case_series · Level IV

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Abstract

The synucleinopathies are neurodegenerative disorders defined by inclusions composed of aberrantly fibrillized alpha-synuclein, but factors contributing to this process remain largely unknown. The authors examined the glucocerebrosidase gene in 75 autopsy specimens with different synucleinopathies and identified mutations in 23% of cases of dementia with Lewy bodies, expanding on previous findings in subjects with Parkinson disease. Mutations in this lysosomal protein may interfere with the clearance or promote aggregation of alpha-synuclein.

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