Proteostasis Therapeutics, developing novel therapeutics that regulate protein homeostasis aimed at helping to improve outcomes for patients with orphan and neurodegenerative diseases, has entered into a worldwide collaboration with Biogen Idec to research and develop therapeutic candidates based on the inhibition of Usp14.

The collaboration combines Proteostasis Therapeutics' proprietary scientific platform and preclinical work on protein degradation with Biogen Idec's neurodegenerative disease research and clinical development capabilities.

Preclinical research has shown the inhibition of Usp14, a deubiquitinating enzyme, modulates proteasome activity and increases the degradation rate of aggregation-prone proteins, including α-synuclein in Parkinson's disease and tau in Alzheimer's disease. The collaboration is intended to develop Usp14 inhibitors as a disease-modifying approach for disorders involving toxic protein aggregation.

Proteostasis will receive an initial upfront payment from Biogen Idec, along with an equity investment, and is eligible for research funding support and future milestones that could result in total payments of up to $200 million, as well as tiered royalties. The companies will conduct preclinical research to identify lead compounds for clinical development. At specified points in development, Proteostasis will have the option to receive potential milestones or opt in for global co-development and U.S. co-commercialization rights.