Article
LYSET facilitates integration of both the N- and C-terminal transmembrane helices/cytoplasmic domains of GlcNAc-1-phosphotransferase.
Molecular biology of the cell - 1 Apr 2025
Doray Baraj, Jennings Benjamin C, Yang Xi, Liu Lin, Venkatarangan Varsha, Kornfeld Stuart, Li Ming
Abstract excerpt
LYSET is a recently identified Golgi transmembrane (TM) protein, and inactivating mutations in the LYSET gene phenocopy mucolipidosis II (MLII), the lysosomal storage disease caused by loss of function of GlcNAc-1-phosphotransferase αβ (GNPTαβ), which tags lysosomal hydrolases with the mannose 6-phosphate (M6P) tag for delivery to lysosomes. It is conceivable that LYSET facilitates integration of both hydrophilic...
Read the complete abstract on PubMedTopics
- Humans
- Golgi Apparatus
- Transferases (Other Substituted Phosphate Groups)
- Protein Domains
- Lysosomes
- Mucolipidoses
