The Batten disease protein CLN8 is a stereospecific acyltransferase in bis(monoacylglycero)phosphate biosynthesis.

CLN8 is a stereospecific acyltransferase essential for BMP biosynthesis, with mutations impairing its activity linked to Batten disease in mice and zebrafish.

• Why it matters: Understanding CLN8’s precise role in BMP production addresses a critical gap in the molecular basis of Batten disease, a devastating neurodegenerative disorder.
• What they did: The study used cryo-electron microscopy and biochemical assays to show that CLN8 catalyzes stereospecific acylation of S,S-glycerophosphoglycerol, with mutations disrupting this process.
• The result: Supplementing with S,S-lysophosphatidylglycerol restores BMP synthesis and neurological function, suggesting a potential therapeutic approach for CLN8-related Batten disease.