Human Ataxin-2 is implicated in the cause and progression of amyotrophic lateral sclerosis (ALS) and type 2 spinocerebellar ataxia (SCA-2). In Drosophila, a conserved atx2 gene is essential for animal survival as well as for normal RNP-granule assembly, translational control, and long-term habituation. Like its human homolog, Drosophila Ataxin-2 (Atx2) contains polyQ repeats and additional intrinsically disordered regions (IDRs). We demonstrate that Atx2 IDRs, which are capable of mediating liquid-liquid phase transitions in vitro, are essential for efficient formation of neuronal mRNP assemblies in vivo. Remarkably, ΔIDR mutants that lack neuronal RNP granules show normal animal development, survival, and fertility. However, they show defects in long-term memory formation/consolidation as well as in C9ORF72 dipeptide repeat or FUS-induced neurodegeneration. Together, our findings demonstrate (1) that higher-order mRNP assemblies contribute to long-term neuronal plasticity and memory, and (2) that a targeted reduction in RNP-granule formation efficiency can alleviate specific forms of neurodegeneration.
Pubmed ID: 29772202 RIS Download
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This monoclonal targets Drosophila FMR1
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View all literature mentionsDrosophila melanogaster with name ry[506] P{ry[+t7.2]=PZ}Atx2[06490]/TM3, ry[RK] Sb[1] Ser[1] from BDSC.
View all literature mentionsDrosophila melanogaster with name w[*]; P{w[+mC]=GAL4-ninaE.GMR}12 from BDSC.
View all literature mentionsCell line S2R+ is a Spontaneously immortalized cell line with a species of origin Drosophila melanogaster (Fruit fly)
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