The Drosophila melanogaster as Genetic Model System to Dissect the Mechanisms of Disease that Lead to Neurodegeneration in Adrenoleukodystrophy

Abstract

Drosophila melanogaster is the most successful genetic model organism to study different human disease with a recent increased popularity to study neurological disorders. Drosophila melanogaster has a complex yet well-defined brain with defined anatomical regions with specific functions. The neuronal network in the adult brain has a structural organization highly similar to human neurons, but in a brain that is much more amenable for complex analyses. The availability of sophisticated genetic tools to study neurons permits to examine neuronal functions at the single cell level in the whole brain by confocal imaging, which does not require sections. Thus, Drosophila has been used to successfully study many neurological disorders such as Parkinson's disease and has been recently adopted to understand the complex networks leading to neurological disorders with metabolic origins such as Leigh disease and X-linked adrenoleukodystrophy (X-ALD).In this review, we will describe the genetic tools available to study neuronal structures and functions and also illustrate some limitations of the system. Finally, we will report the experimental efforts that in the past 10 years have established Drosophila melanogaster as an excellent model organism to study neurodegenerative disorders focusing on X-ALD.