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

CLN8 is a stereospecific glycerophosphoglycerol acyltransferase essential for BMP biosynthesis, with mutations impairing activity linked to Batten disease.

• Why it matters: Understanding CLN8's role clarifies the biochemical basis of Batten disease and offers potential therapeutic avenues for this 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, producing the CLN5 substrate in BMP synthesis, and demonstrated that mutations impair this activity.
• The result: Exogenous S,S-lysophosphatidylglycerol restores BMP production and neurological function in CLN8-deficient models, suggesting BMP precursor supplementation as a promising therapeutic strategy.