A novel inhibitor of the insulin/IGF signaling pathway protects from age-onset, neurodegeneration-linked proteotoxicity

Aging Cell. 2014 Feb;13(1):165-74. doi: 10.1111/acel.12171. Epub 2013 Nov 22.

Abstract

Aging manipulation is an emerging strategy aimed to postpone the manifestation of late-onset neurodegenerative disorders such as Alzheimer's (AD) and Huntington's diseases (HD) and to slow their progression once emerged. Reducing the activity of the insulin/IGF signaling cascade (IIS), a prominent aging-regulating pathway, protects worms from proteotoxicity of various aggregative proteins, including the AD-associated peptide, Aβ- and the HD-linked peptide, polyQ40. Similarly, IGF1 signaling reduction protects mice from AD-like disease. These discoveries suggest that IIS inhibitors can serve as new drugs for the treatment of neurodegenerative maladies including AD and HD. Here, we report that NT219, a novel IIS inhibitor, mediates a long-lasting, highly efficient inhibition of this signaling cascade by a dual mechanism; it reduces the autophosphorylation of the IGF1 receptor and directs the insulin receptor substrates 1 and 2 (IRS 1/2) for degradation. NT219 treatment promotes stress resistance and protects nematodes from AD- and HD-associated proteotoxicity without affecting lifespan. Our discoveries strengthen the theme that IIS inhibition has a therapeutic potential as a cure for neurodegenerative maladies and point at NT219 as a promising compound for the treatment of these disorders through a selective manipulation of aging.

Keywords: C. elegans; aging; insulin/IGF signaling inhibitor; neurodegeneration; proteostasis.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aging / metabolism
  • Aging / pathology*
  • Amyloid beta-Peptides / toxicity*
  • Animals
  • Caenorhabditis elegans / drug effects
  • Caenorhabditis elegans / genetics
  • Caenorhabditis elegans / radiation effects
  • Cell Line, Tumor
  • Gene Expression Regulation / drug effects
  • Hot Temperature
  • Humans
  • Insulin / metabolism*
  • Insulin-Like Growth Factor I / metabolism*
  • Longevity / drug effects
  • Longevity / radiation effects
  • Mice
  • Models, Animal
  • Nerve Degeneration / metabolism
  • Nerve Degeneration / pathology*
  • Neuroprotective Agents / pharmacology*
  • Peptides / toxicity*
  • Proto-Oncogene Proteins c-akt / metabolism
  • Receptor, IGF Type 1 / metabolism
  • Signal Transduction / drug effects
  • Signal Transduction / genetics
  • Signal Transduction / radiation effects
  • Stress, Physiological / drug effects
  • Stress, Physiological / genetics
  • Stress, Physiological / radiation effects
  • Ultraviolet Rays

Substances

  • Amyloid beta-Peptides
  • Insulin
  • Neuroprotective Agents
  • Peptides
  • polyglutamine
  • Insulin-Like Growth Factor I
  • Receptor, IGF Type 1
  • Proto-Oncogene Proteins c-akt