Glycogen Branching Enzyme is an important component in the neurobiology of neurodegenerative diseases. This page provides detailed information about its structure, function, and role in disease processes.
| GBE1 | |
|---|---|
| Glycogen Branching Enzyme | |
| Protein Name | Glycogen Branching Enzyme |
| Gene | [GBE1](/genes/gbe1) |
| UniProt ID | [Q9BZN1](https://www.uniprot.org/uniprot/Q9BZN1) |
| PDB ID | [2VJC](https://www.rcsb.org/structure/2VJC) |
| Molecular Weight | 80 kDa |
| Subcellular Localization | Cytoplasm, Glycogen particle |
| Protein Family | Glycosyltransferase family 48 |
GBE1 (Glycogen Branching Enzyme) is a protein encoded by the GBE1 gene. It is an enzyme that catalyzes the formation of branch points in glycogen by transferring terminal glucose residues from one chain to a hydroxyl group at the C-6 position of an interior glucose residue.
GBE1 is a 702-amino acid protein with:
The glycogen branching enzyme:
Mutations in GBE1 cause accumulation of polyglucosan bodies:
The study of Glycogen Branching Enzyme has evolved significantly over the past decades. Research in this area has revealed important insights into the underlying mechanisms of neurodegeneration and continues to drive therapeutic development.
Historical context and key discoveries in this field have shaped our current understanding and will continue to guide future research directions.