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Zhang, K., Coyne, A.N., Lloyd, T.E. (2018). Drosophila models of amyotrophic lateral sclerosis with defects in RNA metabolism.  Brain Res. 1693(A): 109--120.
FlyBase ID
FBrf0239115
Publication Type
Review
Abstract

The fruit fly Drosophila Melanogaster has been widely used to study neurodegenerative diseases. The conservation of nervous system biology coupled with the rapid life cycle and powerful genetic tools in the fly have enabled the identification of novel therapeutic targets that have been validated in vertebrate model systems and human patients. A recent example is in the study of the devastating motor neuron degenerative disease amyotrophic lateral sclerosis (ALS). Mutations in genes that regulate RNA metabolism are a major cause of inherited ALS, and functional analysis of these genes in the fly nervous system has shed light on how mutations cause disease. Importantly, unbiased genetic screens have identified key pathways that contribute to ALS pathogenesis such as nucleocytoplasmic transport and stress granule assembly. In this review, we will discuss the utilization of Drosophila models of ALS with defects in RNA metabolism.

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    Language of Publication
    English
    Additional Languages of Abstract
    Parent Publication
    Publication Type
    Journal
    Abbreviation
    Brain Res.
    Title
    Brain Research
    Publication Year
    1966-
    ISBN/ISSN
    0006-8993
    Data From Reference