Animal disease models

A fly for Sly

Bar, S., Prasad, M. and Datta, R. Dis. Model Mech. 11, dmm036954 (2018)

Sly syndrome is a recessive lysosomal storage disorder that leads to premature death in humans. It has a mouse model and, as of November 2017, an FDA-approved enzyme therapy. But there’s still much to learn about the mechanisms behind this form of mucopolysaccharidosis and others like it. As an alternative to the mouse, researchers in India recently developed a fly model of Sly syndrome.

The deficient enzyme responsible for the syndrome in humans, β-glucuronidase, has an orthologue in Drosophila melanogaster, CG2135. When that gene is knocked out, the fly experiences similar cardiac problems, motor impairments and neuropathologies, and shortened lifespans. The authors relate those phenotypes to apoptosis in dopaminergic neurons and muscle cells and show that treatment with resveratrol can help attenuate abnormalities in CG2135−/− flies.

Author information



Corresponding author

Correspondence to Ellen P. Neff.

Rights and permissions

Reprints and Permissions

About this article

Verify currency and authenticity via CrossMark

Cite this article

Neff, E.P. A fly for Sly. Lab Anim 48, 46 (2019).

Download citation


Nature Briefing

Sign up for the Nature Briefing newsletter — what matters in science, free to your inbox daily.

Get the most important science stories of the day, free in your inbox. Sign up for Nature Briefing