Expression of human disease alleles in Drosophila used to study cellular mechanisms of diseases associated with polyglutamine expansion

Vu A, Humphries T, Vogel S, Haberman A. Polyglutamine repeat proteins disrupt actin structure in Drosophila photoreceptors. Mol Cell Neurosci. 2018 Aug 24. PMID: 30149064.

The abstract: "Expansions of polygutamine-encoding stretches in several genes cause neurodegenerative disorders including Huntington's Disease and Spinocerebellar Ataxia type 3. Expression of the human disease alleles in Drosophila melanogaster neurons recapitulates cellular features of these disorders, and has therefore been used to model the cell biology of these diseases. Here, we show that polyglutamine disease alleles expressed in Drosophila photoreceptors disrupt actin structure at rhabdomeres, as other groups have shown they do in Drosophila and mammalian dendrites. We show this actin regulatory pathway works through the small G protein Rac and the actin nucleating protein Form3. We also find that Form3 has additional functions in photoreceptors, and that loss of Form3 results in the specification of extra photoreceptors in the eye."