This gene encodes a protein that is essential for nonsense-mediated mRNA decay (NMD)\; a process whereby transcripts with premature termination codons are targeted for rapid degradation by a mRNA decay complex. The mRNA decay complex consists, in part, of this protein along with proteins SMG5 and UPF1. The N-terminal domain of this protein is thought to mediate its association with SMG5 or UPF1 while the C-terminal domain interacts with the mRNA decay complex. This protein may therefore couple changes in UPF1 phosphorylation state to the degradation of NMD-candidate transcripts. Alternative splicing results in multiple transcript variants encoding distinct isoforms. [provided by RefSeq, Aug 2011].
Sakaki, Yoshina, Shen, Han, DeSantis, Xiong, Mitani, Kaufman: "RNA surveillance is required for endoplasmic reticulum homeostasis." in: Proceedings of the National Academy of Sciences of the United States of America, Vol. 109, Issue 21, pp. 8079-84, (2012) (PubMed).