OPA1 helical structures give perspective to mitochondrial dysfunction.

ABSTRACT: Dominant optic atrophy is one of the leading causes of childhood blindness. Around 60-80% of cases¹ are caused by mutations of the gene that encodes optic atrophy protein 1 (OPA1), a protein that has a key role in inner mitochondrial membrane fusion and remodelling of cristae and is crucial for the dynamic organization and regulation of mitochondria². Mutations in OPA1 result in the dysregulation of the GTPase-mediated fusion process of the mitochondrial inner and outer membranes³. Here we used cryo-electron microscopy methods to solve helical structures of OPA1 assembled on lipid membrane tubes, in the presence and absence of nucleotide. These helical assemblies organize into densely packed protein rungs with minimal inter-rung connectivity, and exhibit nu

SUBMITTER: Nyenhuis SB 

PROVIDER: S-EPMC12410014 | biostudies-literature | 2023 Aug

REPOSITORIES: biostudies-literature