ALS Motor-Neuron Screen Supports SIRT1 Activation Across Genotypes

A 512-compound screen in patient-derived motor neurons identified resveratrol tetramers with neuroprotective activity. Follow-up work covered 14 iPSC lines and linked protection to changes in neuronal excitability.

International House at the University of Wollongong, photographed in 2022.
International House at the University of Wollongong, photographed in 2022. (Mx. Granger, CC0 1.0)

Screening Patient-Derived Motor Neurons

A university-led team screened 512 natural products in spinal motor neurons generated from sporadic and familial amyotrophic lateral sclerosis (ALS) donor induced pluripotent stem cells (iPSCs). Live-cell imaging, whole-cell patch clamp and proteomics assessed degeneration, neurite damage and intrinsic hyperexcitability. The library included compounds from fungal, plant and marine-invertebrate sources.

Two resveratrol tetramers, vaticanol B and (−)-hopeaphenol, protected neurons and neurites across the tested genetic backgrounds. Both were more potent than resveratrol in these assays, with (−)-hopeaphenol producing the strongest effects.

Structural Protection and Electrophysiology

In an expanded cohort of 14 iPSC lines spanning sporadic ALS and TARDBP, SOD1 or C9ORF72 backgrounds, (−)-hopeaphenol showed broad cellular protection. It also countered abnormal sodium currents and changes in repetitive firing.

The SIRT1 activator SRT2104 protected neurites, improved ALS-related hyperexcitability and increased neuroprotective pathway activity at one-third of the concentration used for (−)-hopeaphenol. The authors selected it in part for previously described brain permeability and human tolerability; those properties were not new clinical ALS outcomes from this study.

The findings provide preclinical support for a convergent SIRT1-related strategy across the studied genotypes. They combine morphological and electrical measures in patient-derived cells while leaving clinical benefit and disease progression untested.

Sources

  1. Maksour S, et al. Phenotypic screening in ALS motor neurons identifies SIRT1 activation as a genotype-independent neuroprotective strategy. Cell Death & Disease. 2026. doi:10.1038/s41419-026-09384-8.