Article
Triacylglycerol mimetics regulate membrane interactions of glycogen branching enzyme: implications for therapy.
Journal of lipid research - 1 Aug 2017
Alvarez Rafael, Casas Jesús, López David J, Ibarguren Maitane, Suari-Rivera Ariadna, Terés Silvia, Guardiola-Serrano Francisca, Lossos Alexander, Busquets Xavier, Kakhlon Or, Escribá Pablo V
Abstract excerpt
Adult polyglucosan body disease (APBD) is a neurological disorder characterized by adult-onset neurogenic bladder, spasticity, weakness, and sensory loss. The disease is caused by aberrant glycogen branching enzyme (GBE) (GBE1Y329S) yielding less branched, globular, and soluble glycogen, which tends to aggregate. We explore here whether, despite being a soluble enzyme, GBE1 activity is regulated by...
Topics
- Amino Acid Sequence
- Biomimetic Materials
- Cell Membrane
- Enzyme Stability
- Glycogen Debranching Enzyme System
- Glycogen Storage Disease
- Humans
- Mutagenesis, Site-Directed
- Mutation
- Nervous System Diseases
- Protein Binding
- Temperature