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A Drosophila Model for Dentatorubropallidoluysian Atrophy

Final Activity Report Summary - DATROPHIN (A Drosophila Model for Dentatorubropallidoluysian Atrophy)

Dentatorubropallydoluisian atrophy is a neurodegenerative disease caused in humans by the atrophin-1 protein. In the diseased condition a stretch of 14 glutamines is expanded up to 70 by genetic instability of this repeat. The drosophila ortholog of atrophin-1 is atro, a multi-faceted protein involved in several cellular and developmental processes.

During the year covered by the European reintegration grants (ERG) we focussed on studying the role of atro in important signalling pathways and found out that it acted as a negative regulator of the notch signalling pathway by inhibiting activation of transcription by the notch intracellular domain. We also expanded the glutamine stretches in the atro protein in order to mimic the diseased conditions and see if the modified protein retained all the functions of the normal one. Nevertheless, all the transgenic flies we generated expressed very little, if any, modified protein. By the time of the project completion we were analysing all the possible problems, however initial studies suggested that the proteins were quickly degraded by the cells where they were expressed.