TERF2

Telomeric repeat binding factor 2
PDB rendering based on 1h6p.
Available structures PDB Ortholog search: PDBe, RCSB List of PDB id codes 1H6P​, 1VF9​, 1VFC​, 1W0U​, 1XG1​, 3BU8​, 3BUA​, 3K6G​, 3SJM​, 4M7C​
Identifiers
Symbols	TERF2 ; TRBF2; TRF2
External IDs	OMIM: 602027 MGI: 1195972 HomoloGene: 4133 GeneCards: TERF2 Gene
Gene ontology Molecular function • double-stranded telomeric DNA binding • protein binding • protein C-terminus binding • telomeric DNA binding • protein homodimerization activity Cellular component • chromosome, telomeric region • nuclear telomere cap complex • nuclear chromosome, telomeric region • male germ cell nucleus • nucleus • nucleoplasm • cytoplasm • Golgi apparatus • Mre11 complex • telosome Biological process • telomere maintenance • in utero embryonic development • telomere maintenance via telomerase • cell cycle • positive regulation of gene expression • negative regulation of gene expression • telomere capping • telomeric loop formation • protection from non-homologous end joining at telomere • regulation of telomere maintenance • negative regulation of telomere maintenance • positive regulation of telomere maintenance • negative regulation of telomere maintenance via semi-conservative replication • positive regulation of nitric-oxide synthase activity • protein localization to chromosome, telomeric region • negative regulation of cell aging • cellular senescence • negative regulation of beta-galactosidase activity • negative regulation of telomere single strand break repair • negative regulation of cellular senescence Sources: Amigo / QuickGO
RNA expression pattern
More reference expression data
Orthologs
Species	Human	Mouse
Entrez	7014	21750
Ensembl	ENSG00000132604	ENSMUSG00000031921
UniProt	Q15554	O35144
RefSeq (mRNA)	NM_005652	NM_001083118
RefSeq (protein)	NP_005643	NP_001076587
Location (UCSC)	Chr 16: 69.36 – 69.41 Mb	Chr 8: 107.07 – 107.1 Mb
PubMed search

Telomeric repeat-binding factor 2 is a protein that is also known as TRF2 and TRBF2. It is in humans encoded by the TERF2 gene.^[1][2]

Function

This gene encodes a telomere specific protein, TERF2, which is a component of the shelterin nucleoprotein complex. This protein is present at telomeres in metaphase of the cell cycle, is a second negative regulator of telomere length and plays a key role in the protective activity of telomeres. While having similar telomere binding activity and domain organization, TERF2 differs from TERF1 in that its N terminus is basic rather than acidic.^[3]

Interactions

TERF2 has been shown to interact with:

• Ku70,^[4]
• MRE11A,^[5]
• Nibrin^[5] and
• Rad50,^[5][6]
• TERF2IP,^[5][6][7] and
• Werner syndrome ATP-dependent helicase.^[8]

References

1. Broccoli D, Smogorzewska A, Chong L, de Lange T (November 1997). "Human telomeres contain two distinct Myb-related proteins, TRF1 and TRF2". Nat. Genet. 17 (2): 231–5. doi:10.1038/ng1097-231. PMID 9326950.
2. Sakaguchi AY, Padalecki SS, Mattern V, Rodriguez A, Leach RJ, McGill JR, Chavez M, Giambernardi TA (May 1999). "Chromosomal sublocalization of the transcribed human telomere repeat binding factor 2 gene and comparative mapping in the mouse". Somat. Cell Mol. Genet. 24 (3): 157–63. doi:10.1023/B:SCAM.0000007118.47691.d7. PMID 10226653. Check date values in: |year= / |date= mismatch (help)
3. "Entrez Gene: TERF2 telomeric repeat binding factor 2".
4. Song K, Jung D, Jung Y, Lee SG, Lee I (September 2000). "Interaction of human Ku70 with TRF2". FEBS Lett. 481 (1): 81–5. doi:10.1016/s0014-5793(00)01958-x. PMID 10984620.
5. Zhu XD, Küster B, Mann M, Petrini JH, de Lange T (July 2000). "Cell-cycle-regulated association of RAD50/MRE11/NBS1 with TRF2 and human telomeres". Nat. Genet. 25 (3): 347–52. doi:10.1038/77139. PMID 10888888.
6. O'Connor MS, Safari A, Liu D, Qin J, Songyang Z (July 2004). "The human Rap1 protein complex and modulation of telomere length". J. Biol. Chem. 279 (27): 28585–91. doi:10.1074/jbc.M312913200. PMID 15100233.
7. Li B, Oestreich S, de Lange T (May 2000). "Identification of human Rap1: implications for telomere evolution". Cell 101 (5): 471–83. doi:10.1016/S0092-8674(00)80858-2. PMID 10850490.
8. Opresko PL, von Kobbe C, Laine JP, Harrigan J, Hickson ID, Bohr VA (October 2002). "Telomere-binding protein TRF2 binds to and stimulates the Werner and Bloom syndrome helicases". J. Biol. Chem. 277 (43): 41110–9. doi:10.1074/jbc.M205396200. PMID 12181313.

Further reading

• Bilaud T, Koering CE, Binet-Brasselet E, Ancelin K, Pollice A, Gasser SM, Gilson E (1996). "The telobox, a Myb-related telomeric DNA binding motif found in proteins from yeast, plants and human". Nucleic Acids Res. 24 (7): 1294–303. doi:10.1093/nar/24.7.1294. PMC 145771. PMID 8614633.
• Bilaud T, Brun C, Ancelin K, Koering CE, Laroche T, Gilson E (1997). "Telomeric localization of TRF2, a novel human telobox protein". Nat. Genet. 17 (2): 236–9. doi:10.1038/ng1097-236. PMID 9326951.
• van Steensel B, Smogorzewska A, de Lange T (1998). "TRF2 protects human telomeres from end-to-end fusions". Cell 92 (3): 401–13. doi:10.1016/S0092-8674(00)80932-0. PMID 9476899.
• Griffith JD, Comeau L, Rosenfield S, Stansel RM, Bianchi A, Moss H, de Lange T (1999). "Mammalian telomeres end in a large duplex loop". Cell 97 (4): 503–14. doi:10.1016/S0092-8674(00)80760-6. PMID 10338214.
• Teichmann M, Wang Z, Martinez E, Tjernberg A, Zhang D, Vollmer F, Chait BT, Roeder RG (1999). "Human TATA-binding protein-related factor-2 (hTRF2) stably associates with hTFIIA in HeLa cells". Proc. Natl. Acad. Sci. U.S.A. 96 (24): 13720–5. doi:10.1073/pnas.96.24.13720. PMC 24131. PMID 10570139.
• Smogorzewska A, van Steensel B, Bianchi A, Oelmann S, Schaefer MR, Schnapp G, de Lange T (2000). "Control of human telomere length by TRF1 and TRF2". Mol. Cell. Biol. 20 (5): 1659–68. doi:10.1128/MCB.20.5.1659-1668.2000. PMC 85349. PMID 10669743.
• Li B, Oestreich S, de Lange T (2000). "Identification of human Rap1: implications for telomere evolution". Cell 101 (5): 471–83. doi:10.1016/S0092-8674(00)80858-2. PMID 10850490.
• Zhu XD, Küster B, Mann M, Petrini JH, de Lange T (2000). "Cell-cycle-regulated association of RAD50/MRE11/NBS1 with TRF2 and human telomeres". Nat. Genet. 25 (3): 347–52. doi:10.1038/77139. PMID 10888888.
• Song K, Jung D, Jung Y, Lee SG, Lee I (2000). "Interaction of human Ku70 with TRF2". FEBS Lett. 481 (1): 81–5. doi:10.1016/S0014-5793(00)01958-X. PMID 10984620.
• Song K, Jung Y, Jung D, Lee I (2001). "Human Ku70 interacts with heterochromatin protein 1alpha". J. Biol. Chem. 276 (11): 8321–7. doi:10.1074/jbc.M008779200. PMID 11112778.
• Fairall L, Chapman L, Moss H, de Lange T, Rhodes D (2001). "Structure of the TRFH dimerization domain of the human telomeric proteins TRF1 and TRF2". Mol. Cell 8 (2): 351–61. doi:10.1016/S1097-2765(01)00321-5. PMID 11545737.
• Bailey SM, Cornforth MN, Kurimasa A, Chen DJ, Goodwin EH (2001). "Strand-specific postreplicative processing of mammalian telomeres". Science 293 (5539): 2462–5. doi:10.1126/science.1062560. PMID 11577237.
• Karlseder J, Smogorzewska A, de Lange T (2002). "Senescence induced by altered telomere state, not telomere loss". Science 295 (5564): 2446–9. doi:10.1126/science.1069523. PMID 11923537.
• Mignon-Ravix C, Depetris D, Delobel B, Croquette MF, Mattei MG (2002). "A human interstitial telomere associates in vivo with specific TRF2 and TIN2 proteins". Eur. J. Hum. Genet. 10 (2): 107–12. doi:10.1038/sj.ejhg.5200775. PMID 11938440.
• Ancelin K, Brunori M, Bauwens S, Koering CE, Brun C, Ricoul M, Pommier JP, Sabatier L, Gilson E (2002). "Targeting assay to study the cis functions of human telomeric proteins: evidence for inhibition of telomerase by TRF1 and for activation of telomere degradation by TRF2". Mol. Cell. Biol. 22 (10): 3474–87. doi:10.1128/MCB.22.10.3474-3487.2002. PMC 133804. PMID 11971978.
• Opresko PL, von Kobbe C, Laine JP, Harrigan J, Hickson ID, Bohr VA (2002). "Telomere-binding protein TRF2 binds to and stimulates the Werner and Bloom syndrome helicases". J. Biol. Chem. 277 (43): 41110–9. doi:10.1074/jbc.M205396200. PMID 12181313.
• Chagnon P, Michaud J, Mitchell G, Mercier J, Marion JF, Drouin E, Rasquin-Weber A, Hudson TJ, Richter A (2002). "A missense mutation (R565W) in cirhin (FLJ14728) in North American Indian childhood cirrhosis". Am. J. Hum. Genet. 71 (6): 1443–9. doi:10.1086/344580. PMC 378590. PMID 12417987.