MATR3

Cilia effects upon perturbation of MATR3

Loss-of-function effect:

Longer cilia

Subcellular localization

nucleus

References: doi:10.64898/2026.06.09.731172

Function

Matrin-3; nuclear-matrix scaffold and DNA-/RNA-binding protein regulating transcription, RNA stability and splicing, with a cytoplasmic pool associating with the translation machinery. Cilia-associated on functional grounds (Lee et al. 2026): identified as a key driver of a convergent ciliary transcriptional program downstream of schizophrenia common-risk genes; CRISPR perturbation in mouse neocortical cells significantly increased primary cilia length. Mutations cause ALS and distal myopathy with vocal-cord/pharyngeal weakness (VCPDM). Nuclear, not ciliary-localizing; ciliary link is functional (cilia-length phenotype).