Linear ubiquitination is involved in the pathogenesis of optineurin-associated amyotrophic lateral sclerosis

Abstract

Mutations in optineurin are associated with neurodegenerative diseases, including amyotrophic lateral sclerosis. Here, the authors report the structure of the ubiquitin binding domain of optineurin, which binds linear ubiquitin with homology to NEMO, and explore the function of this domain.