Molecular Insights into the Regulation of GNPTAB by TMEM251
Xi Yang, Balraj Doray, Danielle Henn, Varsha Venkatarangan, Benjamin C. Jennings, Zhongzheng Dong, Jiaxuan Liang, Weichao Zhang, Bokai Zhang, Linchen Yu, Liang Chen, Stuart A. Kornfeld, Ming Li
This study identifies TMEM251 as a two-transmembrane protein that stabilizes GNPT, prevents its mislocalization, and ensures M6P pathway fidelity through interactions with GOLPH3 and retromer complexes.
Nature Communications · Mar 10, 2026Peer-reviewedCell Biology