The multifaceted roles of the brain glycogen

Aug 9, 2023Journal of neurochemistry

The many roles of stored sugar in the brain

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Abstract

Glycogen plays critical roles in multiple human diseases, particularly in the brain.

Glycogen is essential for carbohydrate storage and energy metabolism in the liver and muscle.
Recent research highlights glycogen's important metabolic and non-metabolic functions in the brain.
Disruptions in glycogen homeostasis are linked to various neurological glycogen storage diseases (n-GSDs).
n-GSDs, such as Lafora disease and adult polyglucosan body disease, are neurogenetic disorders that can lead to severe neurological symptoms.
Innovative technologies have provided new insights into glycogen functions and their impact on neuronal and glial activities.
Current treatment strategies for n-GSDs are being developed and may offer significant benefits to affected patients.

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Glycogen is a biologically essential macromolecule that is directly involved in multiple human diseases. While its primary role in carbohydrate storage and energy metabolism in the liver and muscle is well characterized, recent research has highlighted critical metabolic and non-metabolic roles for glycogen in the brain. In this review, the emerging roles of glycogen homeostasis in the healthy and diseased brain are discussed with a focus on advancing our understanding of the role of glycogen in the brain. Innovative technologies that have led to novel insights into glycogen functions are detailed. Key insights into how cellular localization impacts neuronal and glial function are discussed. Perturbed glycogen functions are observed in multiple disorders of the brain, including where it serves as a disease driver in the emerging category of neurological glycogen storage diseases (n-GSDs). n-GSDs include Lafora disease (LD), adult polyglucosan body disease (APBD), Cori disease, Glucose transporter type 1 deficiency syndrome (G1D), GSD0b, and late-onset Pompe disease (PD). They are neurogenetic disorders characterized by aberrant glycogen which results in devastating neurological and systemic symptoms. In the most severe cases, rapid neurodegeneration coupled with dementia results in death soon after diagnosis. Finally, we discuss current treatment strategies that are currently being developed and have the potential to be of great benefit to patients with n-GSD. Taken together, novel technologies and biological insights have resulted in a renaissance in brain glycogen that dramatically advanced our understanding of both biology and disease. Future studies are needed to expand our understanding and the multifaceted roles of glycogen and effectively apply these insights to human disease.

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The multifaceted roles of the brain glycogen

Abstract

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