Skip to main content
. Author manuscript; available in PMC: 2015 Aug 17.
Published in final edited form as: Annu Rev Biochem. 2015 Mar 12;84:435–464. doi: 10.1146/annurev-biochem-060614-033955

Table 2. Effects of pharmacological proteostasis network (PN) modifiers on animal models of neurodegenerative disease.

Compound Effect on PN Disease model Effect Reference
Radicicol Activates HSF1 HD mouse brain slices Delays aggregation 64
Geldanamycin Activates HSF1 PD flies Suppresses loss of dopaminergic neurons 135
17-AAG Activates HSF1 SCA3/MJD flies Suppresses compound eye degeneration and SCA3 aggregation, improves survival 134
17-AAG Activates HSF1 HD flies Suppresses degeneration of photoreceptor neurons 134
HSP990 Activates HSF1 HD mice Transiently suppresses aggregation and motor decline 95
Arimoclomol Coactivates HSF1 ALS mice (G93A) Suppresses motor neuron death, rescues hind-limb function, and increases life span 179
NG-094 Coactivates HSF1 Worms expressing polyQ in body wall Reduces aggregation and suppresses motility defects 180
Dexamethasone Increases HSF1 levels HD mice Suppresses aggregation and improves motor performance 97
Dexamethasone Increases HSF1 levels HD flies Suppresses aggregation and improves behavioral phenotypes 97
Geranylgeranyl-acetone Increases HSP70 levels SBMA mice Suppresses aggregation and muscle wasting and improves motor function 181
HSF1A Activates HSF1 SCA3/MJD flies Restores eye morphology, size, and pigment color 137
F1 Activates multiple stress responses Worms expressing polyQ in body wall muscle Suppresses aggregation and movement defects 136
Rapamycin Increases autophagy HD flies Suppresses photoreceptor neuron death 141
CCI-779 (rapamycin ester) Increases autophagy HD mice Reduces striatal aggregation and improves motor function 141
SMER10 Increases autophagy HD flies Suppresses photoreceptor neuron degeneration 146
SMER18 Increases autophagy HD flies Suppresses photoreceptor neuron degeneration 146
SMER28 Increases autophagy HD flies Suppresses photoreceptor neuron degeneration 146
Verapamil Increases autophagy HD zebrafish Reduces aggregation and restores rhodopsin expression 182
Clonidine Increases autophagy HD zebrafish Reduces aggregation and restores rhodopsin expression 182

Abbreviations: ALS, amyotrophic lateral sclerosis; HD, Huntington's disease; HSF1, heat shock factor 1; PD, Parkinsons disease; SBMA, spinobulbar muscular atrophy; polyQ, polyglutamine; SMER, small-molecule enhancer of rapamycin.