Article

Stereospecific GPG acylation by CLN8 drives BMP biosynthesis and its loss leads to Batten disease

2025-12-20

Abstract excerpt

Loss-of-function mutations in the endoplasmic reticulum membrane protein CLN8 cause Batten disease, a neurodegenerative lysosomal storage disorder 1 . Together with the lysosomal enzyme CLN5, CLN8 mediates the biosynthesis of bis(monoacylglycero)phosphate (BMP), a phospholipid essential for lysosomal function and distinguished by its unique S,S stereochemistry 2,3 . However, the role of CLN8 in BMP synthesis ha...

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Literature Corpus work: 31f6ddc9-a0a6-57db-a0ae-7db7d98b9aa1
DOI: 10.64898/2025.12.18.695077

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