Cookies on this website

We use cookies to ensure that we give you the best experience on our website. If you click 'Accept all cookies' we'll assume that you are happy to receive all cookies and you won't see this message again. If you click 'Reject all non-essential cookies' only necessary cookies providing core functionality such as security, network management, and accessibility will be enabled. Click 'Find out more' for information on how to change your cookie settings.

Spinal muscular atrophy (SMA) is a neuromuscular disorder caused by loss of survival motor neuron (SMN) protein. While SMN restoration therapies are beneficial, they are not a cure. We aimed to identify potentially novel treatments to alleviate muscle pathology combining transcriptomics, proteomics, and perturbational data sets. This revealed potential drug candidates for repurposing in SMA. One of the candidates, harmine, was further investigated in cell and animal models, improving multiple disease phenotypes, including lifespan, weight, and key molecular networks in skeletal muscle. Our work highlights the potential of multiple and parallel data-driven approaches for the development of potentially novel treatments for use in combination with SMN restoration therapies.

More information Original publication

DOI

10.1172/jci.insight.149446

Type

Journal article

Publication Date

2021-07-08T00:00:00+00:00

Volume

6

Keywords

Bioinformatics, Drug therapy, Genetic diseases, Muscle Biology, Neuroscience, Animals, Cells, Cultured, Computational Biology, Disease Models, Animal, Drug Repositioning, Gene Expression Profiling, Harmine, Humans, Mice, Muscle, Skeletal, Muscular Atrophy, Spinal, Neuromuscular Agents, Proteomics, Survival of Motor Neuron 1 Protein