AnGes has signed agreement for the regulatory approval, commercialization and supply of Zokinvy (lonafarnib) to Eiger Biopharmaceuticals for the treatment of Hutchinson-Gilford progeria syndrome (HGPS or progeria) and treatment-deficient progeroid laminopathies (PL) in Japan.

A disease-modifying agent, Zokinvy prevents the buildup of defective, farnesylated proteins that form close bonds with the nuclear envelope.

These connections cause cellular instability and premature aging in children and young people with progeria and PL with processing deficiency.

Eiger received exclusive global rights to lonafarnib from Merck (MSD).

According to the latest deal, AnGes will work to obtain and maintain regulatory approval for Zokinvy from Japanese authorities.

The company will also act as an exclusive partner for the delivery and marketing of therapy.

In addition, AnGes will make advance and principal payments of up to $ 1.5 million to Eiger, which will also receive revenue from product sales.

Ai Yamada, President and CEO of AnGes, said: “We are committed to obtaining regulatory approval as soon as possible to deliver Zokinvy to HGPS and PL patients in Japan.

“In parallel with the regulatory approval process, we will make steady progress in preparing to launch the HGPS diagnostic test as part of the development of tests to diagnose genetic diseases in newborns at AnGes Clinical Research Laboratories (ACRL), established last year.

In November 2020, Zokinvy received approval in the United States to reduce the risk of death from HGPS and to treat PL with a deficiency in treatment.

Zokinvy offers a statistically significant benefit for survival in children and young adults with progeria.

It also demonstrated that it reduced deaths by 60% and increased the average survival time by at least 2.5 years in patients with progeria.

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