INTS1 is a component of the Integrator (INT) complex. INT consists of at least 14 subunits and associates with the C-terminal domain of the largest subunit of the RNA polymerase II (RNAPII) and has a role in the regulation of gene expression and RNA processing. Besides mediating the co-transcriptional 3'-end processing of the U-rich small nuclear RNAs (UsnRNAs), INT also functions in RNAPII promoter proximal pause-release and in the processing of enhancer RNAs. Mutations in the INTS1 gene cause a severe neurodevelopmental syndrome [].
