9g8q

Structural highlights

Disease

SKI2_HUMAN Syndromic diarrhea. The disease is caused by variants affecting the gene represented in this entry.

Function

SKI2_HUMAN Helicase component of the SKI complex, a multiprotein complex that assists the RNA-degrading exosome during the mRNA decay and quality-control pathways (PubMed:16024656, PubMed:32006463, PubMed:35120588). The SKI complex catalyzes mRNA extraction from 80S ribosomal complexes in the 3'-5' direction and channels mRNA to the cytosolic exosome for degradation (PubMed:32006463, PubMed:35120588). SKI-mediated extraction of mRNA from stalled ribosomes allow binding of the Pelota-HBS1L complex and subsequent ribosome disassembly by ABCE1 for ribosome recycling (PubMed:32006463). In the nucleus, the SKI complex associates with transcriptionally active genes in a manner dependent on PAF1 complex (PAF1C) (PubMed:16024656).^{[1] [2] [3]}

References

1. ↑ Zhu B, Mandal SS, Pham AD, Zheng Y, Erdjument-Bromage H, Batra SK, Tempst P, Reinberg D. The human PAF complex coordinates transcription with events downstream of RNA synthesis. Genes Dev. 2005 Jul 15;19(14):1668-73. PMID:16024656 doi:http://dx.doi.org/10.1101/gad.1292105
2. ↑ Zinoviev A, Ayupov RK, Abaeva IS, Hellen CUT, Pestova TV. Extraction of mRNA from Stalled Ribosomes by the Ski Complex. Mol Cell. 2020 Mar 19;77(6):1340-1349.e6. PMID:32006463 doi:10.1016/j.molcel.2020.01.011
3. ↑ Kögel A, Keidel A, Bonneau F, Schäfer IB, Conti E. The human SKI complex regulates channeling of ribosome-bound RNA to the exosome via an intrinsic gatekeeping mechanism. Mol Cell. 2022 Feb 17;82(4):756-769.e8. PMID:35120588 doi:10.1016/j.molcel.2022.01.009