Sorted

Proper labelling in a sorting office ensures post is delivered correctly. In the cell’s own protein and lipid sorting centre, the Golgi apparatus, the enzymes Hip14 and Hip14L busily tag proteins for proper distribution. These enzymes are linked to Huntington’s disease, and researchers have now shown they act as rate-limiting factors for the maturation and fusion of lysosomes, cellular structures that contain degradative enzymes. In fruit flies, this tagging by Hip14 and Patsas, a fruit fly equivalent of Hip14L, was necessary for lysosomes (pictured, green) to mature, become acidic and fuse with secretory granules (red). Without these enzymes, lysosomes were smaller and less functional, and fusion was impaired. In fly neurons, this caused progressive motor decline. Boosting lysosomal fusion rescued these defects, raising the possibility that enhancing lysosome function could help counteract Huntington’s disease–related cellular dysfunction.

Written by Anthony Lewis

Anthony Lewis

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Ant was the web editor in the early days of BPoD, and has since worked with the UK’s top science organisations as a writer and multimedia producer. He now splits his time between freelance work and managing multimedia at The Lancet.

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• Image from work by Győző Szenci and colleagues
• Department of Anatomy, Cell- and Developmental Biology, Eötvös Loránd University, Budapest, Hungary
• Image originally published with a Creative Commons Attribution 4.0 International (CC BY 4.0)
• Published in PLOS Genetics, December 2025