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Table 1 Therapeutic strategies tested in Drosophila model of polyQ diseases

From: Alleviating neurodegeneration in Drosophila models of PolyQ diseases

Therapeutic strategy

Functional mechanism

Drosophila model

Therapeutic result

Reference

RNAi

Inhibiting formation of polyQ-expanded protein

SCA1, SCA3, HD

Positive

Mallik et al. [50]

Cystamine

reducing Huntingtin (Htt) aggregation

HD

Positive

Apostol et al. [51]

Agrawal et al. [52]

Bortvedt et al. [53]

Methylene blue (MB)

Inhibiting Htt aggregation

HD

Positive

Sontag et al [54]

18b-glycyrrhetinic acid

Inhibiting Htt aggregation

HD

Positive

Schulte et al. [55]

Camptothecins

Inhibiting Htt aggregation

HD

Positive

Schulte et al. [55]

OH-camptothecin

Inhibiting Htt aggregation

HD

Positive

Schulte et al. [55]

Carbenoxolone

Inhibiting Htt aggregation

HD

Positive

Schulte et al. [55]

Polyglutamine binding peptide 1 (QBP1)

Inhibiting polyQ aggregation

SCA3/MJD

Positive

Nagai et al. [56]

P42

Inhibiting polyQ-hHtt aggregation

HD

Positive

Arribat et al. [57]

Hsp70

Reversing toxic structure of polyQ-expanded proteins

SCA3/MJD, SBMA

Positive

Warrick et al [58]

Wang et al [59]

Hsp40

Reversing toxic structure of polyQ-expanded proteins

HD

Positive

Kazemi et al [49]

Kuo et al. [60]

Hsp110

Reversing toxic structure of polyQ-expanded proteins

HD

Positive

Kuo et al. [60]

Hsp22

Reversing toxic structure of polyQ-expanded proteins

SCA3/MJD

Positive

Li et al. [61]

Rapamycin

Accelerating toxic proteins degradation

HD

Positive but various side-effects on human

Ravikumar et al. [62]

Morrisett et al. [63]

Letavernier et al. [64]

Kuypers et al. [65]

Maroto et al. [66]

Lithium chloride (LiCl)

Accelerating toxic proteins degradation

SCA3/MJD

Positive

Jia et al. [67]

Normal ataxin-3

Accelerating toxic proteins degradation

SCA3/MJD

Positive

Warrick et al. [68]

Hsp104

Accelerating toxic proteins degradation

SCA3/MJD

Positive

Cushman et al. [69]

Sodium butyrate

Transcriptional regulation

HD

Positive

Steffan et al. [70]

Suberoylanilide hydroxamic acid (SAHA)

Transcriptional regulation

HD

Positive

Steffan et al. [70]

Trichostatin A (TSA)

Transcriptional regulation

SCA3/MJD

Positive

Jung et al. [71]

Valproic acid (VPA)

Transcriptional regulation

SCA3/MJD

Positive

Yi et al. [72]

Myc

Transcriptional regulation

SCA3/MJD

Positive

Singh et al. [73]

Uncoupling proteins (UCPs)

Ameliorating mitochondrial dysfunctions

HD

Positive

Besson et al. [74]

Meclizine

Ameliorating mitochondrial dysfunctions

HD

Positive

Gohil et al. [75]