Article
Interaction with ERp57 is required for progranulin protection against Type 2 Gaucher disease.
Bioscience trends - 15 May 2023
Liu Yuzhao, Zhao Xiangli, Jian Jinlong, Hasan Sadaf, Liu Chuanju
Abstract excerpt
Gaucher disease (GD), one of the most common lysosomal storage diseases, is caused by GBA1 mutations resulting in defective glucocerebrosidase (GCase) and consequent accumulation of its substrates β-glucosylceramide (β-GlcCer). We reported progranulin (PGRN), a secretary growth factor-like molecule and an intracellular lysosomal protein was a crucial co-factor of GCase. PGRN binds to GCase and recruits Heat Shock...
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