Immune System Modulator in Development for ALS

by Amy Madsen on Wed, 2013-12-11 14:19

ImStar Therapeutics is developing a small-molecule drug, IMS-088, for the treatment of amyotrophic lateral sclerosis; the drug's target is a key inflammatory pathway

Article Highlights:
  • ImStar Therapuetics will develop IMS-088 as a potential therapy for amyotrophic lateral sclerosis (ALS).
  • A small-molecule compound, IMS-088 is designed to target an immune system pathway known as TANA (TDP43 Associated NF-kappa B Activation); malfunction of the immune system is thought to contribute to the complex ALS process.
  • In preclinical ALS animal research models, treatment with IMS-088 has proven safe.

ImStar Therapuetics will develop IMS-088 as a potential therapy for amyotrophic lateral sclerosis (ALS), the company announced in a Nov. 26, 2013 press release.

A small-molecule compound, IMS-088 is designed to target an immune system pathway known as TANA (TDP43 Associated NF-kappa B Activation). TDP43 protein is associated with ALS, and malfunction of the immune system is thought to contribute to the complex ALS process.

IMS-088 targets immune system activation 

ImStar Co-founder and Chief Scientific Officer Jean-Pierre Julien, who has received MDA funding for ALS research, has shown that in ALS, TDP43 protein activates the NF-kappa B inflammation pathway. This triggers an exaggerated immune-system response that leads to inflammation and destruction of nerve cells.  

Inhibition of the NF-kappa B pathway in mouse models of ALS produced substantial improvements in motor function and extended survival, indicating that NF-kappa B may be an important new target in ALS drug discovery.

IMS-088, a TANA inhibitor, is designed to inhibit activation of the NF-kappa B pathway. It's derived from withaferin A, a naturally occurring chemical compound found in the leaves of the winter cherry plant.

IMS-088 is safe in preclinical studies

In preclinical ALS animal research models, treatment with IMS-088 has proven safe.

ImStar has filed a patent application with the U.S. Patent and Trademark Office covering IMS-088 and other similar therapeutics.

For more on the immune system in ALS, see the following ALSN articles:

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