Batten disease protein CLN8 enables a non-canonical phospholipid synthesis pathway.

CLN8 enables a non-canonical phospholipid synthesis pathway crucial for lysosomal lipid homeostasis, with deficiency causing lipid accumulation in Batten disease models.

• Why it matters: Understanding alternative lipid synthesis pathways is vital because disruptions can lead to lysosomal storage disorders like Batten disease, impacting cellular function and health.
• What they did: The study used tracer experiments and genetic models to show that CLN8 catalyzes the acylation of glycerophosphoglycerol, producing lysophosphatidylglycerol, which is then converted into BMP, a key lysosomal lipid.
• The result: Loss of CLN8 impairs BMP synthesis, causing lipid buildup in lysosomes, which advances knowledge of lysosomal lipid metabolism and suggests new targets for treating related neurodegenerative diseases.