The chaperonin TRiC component Cct3 is required for axonal transport, myelination, and neuromuscular junction refinement.

ABSTRACT: TRiCopathies are recently discovered neurodevelopmental diseases caused by pathogenic variants in components of the chaperonin tailless complex polypeptide 1 ring complex (TRiC). Composed of chaperonin containing TCP1 subunits 1-8 (CCT1-8), TRiC acts as a chaperone that is required for folding of 10% of proteome, including actin and tubulin. Patients with TRiCopathies display variable combinations of cognitive impairment, epilepsy, polymicrogyria, white matter reduction, cerebellar hypoplasia and alterations in the peripheral neuromuscular system. Here, we aimed at better understanding the pathophysiological role of Cct3 in neurodevelopment, particularly in myelin formation and the neuromuscular system using zebrafish as a model system. We have generated two CRISPR/Cas9 loss-of-function al

SUBMITTER: Zhang X 

PROVIDER: S-EPMC12921322 | biostudies-literature | 2026 Feb

REPOSITORIES: biostudies-literature