EST1A Component of the telomerase ribonucleoprotein (RNP) complex that is essential for the replication of chromosome termini. May have a general role in telomere regulation. Promotes in vitro the ability of TERT to elongate telomeres. Overexpression induces telomere uncapping, chromosomal end-to-end fusions (telomeric DNA persists at the fusion points) and did not perturb TRF2 telomeric localization. Binds to the single-stranded 5'-(GTGTGG)(4)GTGT-3' telomeric DNA, but not to a telomerase RNA template component (TER). Plays a role in nonsense-mediated mRNA decay. Is thought to provide a link to the mRNA degradation machinery as it has endonuclease activity required to initiate NMD, and to serve as an adapter for UPF1 to protein phosphatase 2A (PP2A), thereby triggering UPF1 dephosphorylation. Degrades single-stranded RNA (ssRNA), but not ssDNA or dsRNA. Ubiquitous. 3 alternatively spliced human isoforms have been reported. Note: This description may include information from UniProtKB.
Protein type: DNA-binding; EC 3.1.-.-; Hydrolase; Nucleolus
Chromosomal Location of mouse Ortholog: 11|11 B5
Cellular Component:  chromosome; chromosome, telomeric region; cytoplasm; cytosol; exon-exon junction complex; nucleolus; nucleus; telomerase holoenzyme complex
Molecular Function:  DNA binding; DNA polymerase binding; endonuclease activity; hydrolase activity; metal ion binding; nuclease activity; ribonucleoprotein complex binding; RNA binding; RNA endonuclease activity; telomerase RNA binding; telomeric DNA binding
Biological Process:  negative regulation of telomere capping; nuclear-transcribed mRNA catabolic process, nonsense-mediated decay; regulation of telomere maintenance
Reference #:  P61406 (UniProtKB)
Alt. Names/Synonyms: AI317223; AU041178; EST1-like protein A; Est1a; Ever shorter telomeres 1A; Kiaa0732; MGC7717; OTTMUSP00000006490; Smg-6 homolog; Smg-6 homolog, nonsense mediated mRNA decay factor (C. elegans); Smg6; Telomerase subunit EST1A; Telomerase-binding protein EST1A
Gene Symbols: Smg6
Molecular weight: 160,496 Da
Basal Isoelectric point: 6.38  Predict pI for various phosphorylation states
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Protein Structure Not Found.

Cross-references to other databases:  AlphaFold  |  STRING  |  Reactome  |  BioGPS  |  Pfam  |  ENZYME  |  Phospho.ELM  |  NetworKIN  |  UniProtKB  |  Entrez-Gene  |  GenPept  |  Ensembl Gene  |  Ensembl Protein