A Drosophila model of prion disease and its metabolic changes in the brain.

ABSTRACT:

Background

Prion diseases (PrDs) are fatal transmissible neurodegenerative disorders caused by misfolded prion protein, which is highly expressed in the brain. Drosophila has been employed as a model system for studying mammalian neurodegenerative diseases.

Methods

Drosophila transgenic for hamster prion protein (HaPrP) was generated by Valium20 transformation. Locomotion, longevity, protease resistance, and histology were assessed, and nontargeted metabolomics analyses were performed to investigate the changes in Drosophila metabolism with the HaPrP expression and metformin treatment.

Results

The Drosophila model exhibited pan-neuronal expression of HaPrP, with expression levels increasing with age. Flies displayed reduced climbing ability, shortened lifespan, and va

SUBMITTER: Wang D 

PROVIDER: S-EPMC12464878 | biostudies-literature | 2025 Aug

REPOSITORIES: biostudies-literature