Publications

Integrated Stress Response

The integrated stress response: From mechanism to disease. Science Review 368, 6489: Costa-Mattioli M & Walter P (2020).
Protein misfolding in the endoplasmic reticulum as a conduit to human disease. Nature 529, 326-335. Wang M. & Kaufman R.J.,(2016).
The unfolded protein response in neurodegenerative disorders – therapeutic modulation of the PERK pathway. FEBS J. doi: 10.1111/febs.14422. Hughes D. & Mallucci G.R., (2018).
Preventing proteostasis diseases by selective inhibition of a phosphatase regulatory subunit. Science 348, 239-242. Das I. et al, (2015).
Pharmaceutical integrated stress response enhancement protects oligodendrocytes and provides a potential multiple sclerosis therapeutic. Nature Commun. DOI: 10.1038/ncomms7532. 2015: Way SW. et al, (2015).

ALS

The unfolded protein response in amyotrophic later sclerosis: results of a phase 2 trial. Brain Dalla Bella et al, (2021).
Preventing proteostasis diseases by selective inhibition of a phosphatase regulatory subunit. Science 348, 239-242.Das I. et al, (2015).
Guanabenz, which enhances the unfolded protein response, ameliorates mutant SOD1-induced amyotrophic lateral sclerosis. Neurobiol Dis. Wang et al, (2014).
Guanabenz delays the onset of disease symptoms, extends lifespan, improves motor performance and attenuates motor neuron loss in the SOD1 G93A mouse model of amyotrophic lateral sclerosis. Neuroscience Jiang et al, (2014).
Pharmacological reduction of ER stress protects against TDP-43 neuronal toxicity in vivo. Neurobiol Dis. Vaccaro et al, (2013).

CMT

Treatment with IFB-088 improves neuropathy in CMT1A and CMT1B mice. Mol Neurobiol. 59(7), 4159-4178. Bai Y. et al, (2022).
Preventing proteostasis diseases by selective inhibition of a phosphatase regulatory subunit. Science 348, 239-242. Das I. et al, (2015).
Pharmaceutical integrated stress response enhancement protects oligodendrocytes and provides a potential multiple sclerosis therapeutic. Nature Commun. DOI: 10.1038/ncomms7532. Way SW. et al, (2015).
Charcot-Marie-Tooth disease: an overview of genotypes, phenotypes, and clinical management strategies. PM&R 6, 342-355. El-Abassi R. et al, (2014).
Resetting translational homeostasis restores myelination in Charcot-Marie-Tooth disease type 1B mice. J. Exp. Med. 210(4), 821-838. D’Antonio M. et al, (2013).

Prolonging the integrated stress response enhances CNS remyelination in an inflammatory environment. eLife 2021;10:e65469. Popko B. et al, (2021).
Sephin 1, which prolongs the integrated stress response, is a promising therapeutic for multiple sclerosis. Brain doi:10.1093/brain/awy322. Chen Y. et al, (2019).
Pharmaceutical integrated stress response enhancement protects oligodendrocytes and provides a potential multiple sclerosis therapeutic. Nature Commun. DOI: 10.1038/ncomms7532. Way SW. et al, (2015).