This gene encodes a component of the telomerase ribonucleoprotein complex responsible for the replication and maintenance of chromosome ends. The encoded protein also plays a role in the nonsense-mediated mRNA decay (NMD) pathway, providing the endonuclease activity near the premature translation termination codon that is needed to initiate NMD. Alternatively spliced transcript variants encoding distinct protein isoforms have been described. [provided by RefSeq, Feb 2014]
Schmidt, SA; Foley, PL; et al. Identification of SMG6 cleavage sites and a preferred RNA cleavage motif by global analysis of endogenous NMD targets in human cells. NUCLEIC ACIDS RESEARCH 43:309-323(2015).
Wilusz, JE; Wilusz, J; et al. Nonsense-mediated RNA decay: at the 'cutting edge' of regulated snoRNA production. GENES & DEVELOPMENT 28:2447-2449(2014).