Complement drives glucosylceramide accumulation and tissue inflammation in Gaucher disease

Nature64.80
Volume: 543, Issue: 7643, Pages: 108 - 112
Published: Mar 2, 2017
Abstract
Gaucher disease is caused by mutations in GBA1, which encodes the lysosomal enzyme glucocerebrosidase (GCase). GBA1 mutations drive extensive accumulation of glucosylceramide (GC) in multiple innate and adaptive immune cells in the spleen, liver, lung and bone marrow, often leading to chronic inflammation. The mechanisms that connect excess GC to tissue inflammation remain unknown. Here we show that activation of complement C5a and C5a receptor...
Paper Details
Title
Complement drives glucosylceramide accumulation and tissue inflammation in Gaucher disease
Published Date
Mar 2, 2017
Journal
Volume
543
Issue
7643
Pages
108 - 112
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