Dataset Information
CryoEM structure of human LARGE1 bound to UDP-GlcA
Ontology highlight
ABSTRACT:
SUBMITTER: Nicholas Spellmon
PROVIDER: EMPIAR-12959 | biostudies-other |
REPOSITORIES: biostudies-other
Publications
LARGE1 processively polymerizes length-controlled matriglycan on prodystroglycan.
Nature communications 20251010 1
Matriglycan is a linear glycan (xylose-β1,3-glucuronate)<sub>n</sub>, which binds proteins in the extracellular matrix that contain laminin-globular domains and Lassa Fever Virus. It is indispensable for neuromuscular function. Matriglycan of insufficient length can cause muscular dystrophy with abnormal brain and eye development. LARGE1 (Like-acetylglucosaminyltransferase-1) uniquely synthesizes matriglycan on dystroglycan. The mechanism of matriglycan synthesis is not obvious from cryo-EM reco ...[more]
