PUBLICATION
Trehalose Ameliorates Zebrafish Emotional and Social Deficits Caused by CLN8 Dysfunction
- Authors
- Licitra, R., Della Vecchia, S., Santucci, L., Vivarelli, R., Bernardi, S., Santorelli, F.M., Marchese, M.
- ID
- ZDB-PUB-250111-3
- Date
- 2025
- Source
- Cells 14(1): (Journal)
- Registered Authors
- Santorelli, Filippo Maria
- Keywords
- CLN8, behavior, dietary treatment, neuronal ceroid lipofuscinoses, translational medicine, trehalose, zebrafish
- MeSH Terms
-
- Disease Models, Animal
- Zebrafish*
- Anxiety/drug therapy
- Neuronal Ceroid-Lipofuscinoses*/drug therapy
- Neuronal Ceroid-Lipofuscinoses*/genetics
- Social Behavior
- Animals
- Membrane Proteins/genetics
- Membrane Proteins/metabolism
- Locomotion/drug effects
- Emotions/drug effects
- Trehalose*/pharmacology
- Trehalose*/therapeutic use
- Behavior, Animal/drug effects
- Zebrafish Proteins/genetics
- Zebrafish Proteins/metabolism
- PubMed
- 39791756 Full text @ Cells
Citation
Licitra, R., Della Vecchia, S., Santucci, L., Vivarelli, R., Bernardi, S., Santorelli, F.M., Marchese, M. (2025) Trehalose Ameliorates Zebrafish Emotional and Social Deficits Caused by CLN8 Dysfunction. Cells. 14(1):.
Abstract
CLN8 and other neuronal ceroid lipofuscinoses (NCLs) often lead to cognitive decline, emotional disturbances, and social deficits, worsening with disease progression. Disrupted lysosomal pH, impaired autophagy, and defective dendritic arborization contribute to these symptoms. Using a cln8-/- zebrafish model, we identified significant impairments in locomotion, anxiety, and aggression, along with subtle deficits in social interactions, positioning zebrafish as a useful model for therapeutic studies in NCL. Our findings show that trehalose, an autophagy enhancer, ameliorates anxiety, and modestly improves social behavior and predator avoidance in mutant zebrafish. This finding aligns animal models with clinical reports suggestive of behavioral improvements in NCL patients. Trehalose holds promise as a therapeutic agent for CLN8, warranting further research into its neuroprotective mechanisms and clinical applications.
Genes / Markers
Expression
Phenotype
Mutations / Transgenics
Human Disease / Model
Sequence Targeting Reagents
Fish
Orthology
Engineered Foreign Genes
Mapping