The Batten disease gene product CLN6 is a glycerophosphoglycerol epimerase that establishes S,S-BMP stereochemistry
Zhijun Zhu, Jian Xiong, Ibrahim B Maaz, Matthew J. Matrongolo, Ali Ghoochani, Chetan Chilkunda, Xuejun Peng, Anish Alur, Christian Porsen, Angela Schulz, Susan Cotman, Natalia Gomez‐Ospina, Monther Abu-Remaileh
This study identifies CLN6 as a glycerophosphoglycerol epimerase that converts R,S-GPG to S,S-GPG, establishing the S,S-BMP stereochemistry critical for lysosomal function.
bioRxiv · Oct 9, 2026 · PreprintBiochemistry