The Batten disease protein CLN... Note

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

Two studies show that the endoplasmic reticulum-associated protein CLN8 catalyses the acyl CoA-dependent acylation of glycerophosphoglycerol, producing lysophosphatidylglycerol, which is then converted into bis(monoacylglycero)phosphate. These results provide insight into CLN8 Batten disease.