Impaired ribosome-associated quality control of C9orf72 arginine-rich dipeptide-repeat proteins.

ABSTRACT: Protein quality control pathways have evolved to ensure the fidelity of protein synthesis and efficiently clear potentially toxic protein species. Defects in ribosome-associated quality control and its associated factors have been implicated in the accumulation of aberrant proteins and neurodegeneration. C9orf72 repeat-associated non-AUG translation has been suggested to involve inefficient translation elongation, lead to ribosomal pausing and activation of ribosome-associated quality control pathways. However, the role of the ribosome-associated quality control complex in the processing of proteins generated through this non-canonical translation is not well understood. Here we use reporter constructs containing the C9orf72-associated hexanucleotide repeat, ribosome-associated quality con

SUBMITTER: Viera Ortiz AP 

PROVIDER: S-EPMC10316761 | biostudies-literature | 2023 Jul

REPOSITORIES: biostudies-literature