Small molecule C381 targets the lysosome to reduce inflammation and ameliorate disease in models of neurodegeneration

Proc Natl Acad Sci U S A. 2022 Mar 15;119(11):e2121609119. doi: 10.1073/pnas.2121609119. Epub 2022 Mar 8.

Abstract

SignificanceNeurodegenerative diseases are poorly understood and difficult to treat. One common hallmark is lysosomal dysfunction leading to the accumulation of aggregates and other undegradable materials, which cause damage to brain resident cells. Lysosomes are acidic organelles responsible for breaking down biomolecules and recycling their constitutive parts. In this work, we find that the antiinflammatory and neuroprotective compound, discovered via a phenotypic screen, imparts its beneficial effects by targeting the lysosome and restoring its function. This is established using a genome-wide CRISPRi target identification screen and then confirmed using a variety of lysosome-targeted studies. The resulting small molecule from this study represents a potential treatment for neurodegenerative diseases as well as a research tool for the study of lysosomes in disease.

Keywords: drug development; drug discovery; inflammation; lysosomes; neurodegenerative disease.

MeSH terms

  • Animals
  • Anti-Inflammatory Agents / chemistry
  • Anti-Inflammatory Agents / pharmacology*
  • Biomarkers
  • Brain / drug effects
  • Brain / metabolism
  • Disease Models, Animal
  • Disease Susceptibility
  • Drug Development
  • Gene Expression Profiling
  • Humans
  • Lysosomes / drug effects*
  • Mice
  • Neurodegenerative Diseases / drug therapy
  • Neurodegenerative Diseases / etiology
  • Neurodegenerative Diseases / metabolism*
  • Neurodegenerative Diseases / pathology
  • Neurons / drug effects
  • Neurons / metabolism
  • Smad Proteins / agonists

Substances

  • Anti-Inflammatory Agents
  • Biomarkers
  • Smad Proteins