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Author

Jian Xiong

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Open access Oct 2026

Lysosomal lipidomics identifies glycosylated bis(monoacylglycero)phosphate as a novel biomarker of mutant GBA1

Mutations in GBA1, which encodes the lysosomal enzyme glucocerebrosidase (GCase), cause Gaucher disease (GD) and represent a major genetic risk factor for Parkinson’s disease (PD). PD clinical trials targeting or monitoring GCase canonical substrates (Glucosyl-ceramides/sphingosine) have failed to achieve their targets...

Cindy Lin, Miharu Iguchi, Jacob Oh et al. · 0 citations
Open access Oct 2026

The Batten disease gene product CLN6 is a glycerophosphoglycerol epimerase that establishes S,S-BMP stereochemistry

Bis(monoacylglycero)phosphate (BMP), a lysosomal phospholipid essential for lipid catabolism and cholesterol clearance, has a noncanonical S,S glycerol configuration found in no other mammalian phospholipid, a feature necessary for its stability in the lysosome. How cells invert glycerol stereochemistry to build S,S-BM...

Zhi-Jun Zhu, Jian Xiong, Ibrahim B. Maaz et al. · 0 citations

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