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TERF2 / TRF2

telomeric repeat binding factor 2

Binds the telomeric double-stranded 5'-TTAGGG-3' repeat and plays a central role in telomere maintenance and protection against end-to-end fusion of chromosomes. In addition to its telomeric DNA-binding role, required to recruit a number of factors and enzymes required for telomere protection, including the shelterin complex, TERF2IP/RAP1 and DCLRE1B/Apollo. Component of the shelterin complex (telosome) that is involved in the regulation of telomere length and protection. Shelterin associates with arrays of double-stranded 5'-TTAGGG-3' repeats added by telomerase and protects chromosome ends; without its protective activity, telomeres are no longer hidden from the DNA damage surveillance and chromosome ends are inappropriately processed by DNA repair pathways. Together with DCLRE1B/Apollo, plays a key role in telomeric loop (T loop) formation by generating 3' single-stranded overhang at the leading end telomeres: T loops have been proposed to protect chromosome ends from degradation and repair. Required both to recruit DCLRE1B/Apollo to telomeres and activate the exonuclease activity of DCLRE1B/Apollo. Preferentially binds to positive supercoiled DNA. Together with DCLRE1B/Apollo, required to control the amount of DNA topoisomerase (TOP1, TOP2A and TOP2B) needed for telomere replication during fork passage and prevent aberrant telomere topology. Recruits TERF2IP/RAP1 to telomeres, thereby participating in to repressing homology-directed repair (HDR), which can affect telomere length.

Gene Name: telomeric repeat binding factor 2
Synonyms: TERF2, TRF2, TRBF2, TTAGGG repeat-binding factor 2, Telomeric DNA-binding protein
Target Sequences: NM_005652 NP_005643.2 Q15554

Publications (25)

1
Control of human telomere length by TRF1 and TRF2. Smogorzewska A, van Steensel B, Bianchi A, Oelmann S, Schaefer MR, Schnapp G, de Lange T. Molecular and cellular biology. 2000 20:1659-68. [PubMed:10669743] [PMC:PMC85349]
2
Cell-cycle-regulated association of RAD50/MRE11/NBS1 with TRF2 and human telomeres. Zhu XD, Kster B, Mann M, Petrini JH, de Lange T. Nature genetics. 2000 25:347-52. [PubMed:10888888]
3
Tankyrase promotes telomere elongation in human cells. Smith S, de Lange T. Current biology : CB. 2000 10:1299-302. [PubMed:11069113]
4
Different telomere damage signaling pathways in human and mouse cells. Smogorzewska A, de Lange T. The EMBO journal. 2002 21:4338-48. [PubMed:12169636] [PMC:PMC126171]
5
POT1 as a terminal transducer of TRF1 telomere length control. Loayza D, De Lange T. Nature. 2003 423:1013-8. [PubMed:12768206]
6
DNA damage foci at dysfunctional telomeres. Takai H, Smogorzewska A, de Lange T. Current biology : CB. 2003 13:1549-56. [PubMed:12956959]
7
Rap1 affects the length and heterogeneity of human telomeres. Li B, de Lange T. Molecular biology of the cell. 2003 14:5060-8. [PubMed:14565979] [PMC:PMC284807]
8
ERCC1/XPF removes the 3' overhang from uncapped telomeres and represses formation of telomeric DNA-containing double minute chromosomes. Zhu XD, Niedernhofer L, Kuster B, Mann M, Hoeijmakers JH, de Lange T. Molecular cell. 2003 12:1489-98. [PubMed:14690602]
9
TIN2 is a tankyrase 1 PARP modulator in the TRF1 telomere length control complex. Ye JZ, de Lange T. Nature genetics. 2004 36:618-23. [PubMed:15133513]
10
The telomeric protein TRF2 binds the ATM kinase and can inhibit the ATM-dependent DNA damage response. Karlseder J, Hoke K, Mirzoeva OK, Bakkenist C, Kastan MB, Petrini JH, de Lange T. PLoS biology. 2004 2:E240. [PubMed:15314656] [PMC:PMC509302]
11
TIN2 binds TRF1 and TRF2 simultaneously and stabilizes the TRF2 complex on telomeres. Ye JZ, Donigian JR, van Overbeek M, Loayza D, Luo Y, Krutchinsky AN, Chait BT, de Lange T. The Journal of biological chemistry. 2004 279:47264-71. [PubMed:15316005]
12
Human Rif1, ortholog of a yeast telomeric protein, is regulated by ATM and 53BP1 and functions in the S-phase checkpoint. Silverman J, Takai H, Buonomo SB, Eisenhaber F, de Lange T. Genes & development. 2004 18:2108-19. [PubMed:15342490] [PMC:PMC515289]
13
Homologous recombination generates T-loop-sized deletions at human telomeres. Wang RC, Smogorzewska A, de Lange T. Cell. 2004 119:355-68. [PubMed:15507207]
14
Significant role for p16INK4a in p53-independent telomere-directed senescence. Jacobs JJ, de Lange T. Current biology : CB. 2004 14:2302-8. [PubMed:15620660]
15
DNA processing is not required for ATM-mediated telomere damage response after TRF2 deletion. Celli GB, de Lange T. Nature cell biology. 2005 7:712-8. [PubMed:15968270]
16
POT1 protects telomeres from a transient DNA damage response and determines how human chromosomes end. Hockemeyer D, Sfeir AJ, Shay JW, Wright WE, de Lange T. The EMBO journal. 2005 24:2667-78. [PubMed:15973431] [PMC:PMC1176460]
17
Apollo, an Artemis-related nuclease, interacts with TRF2 and protects human telomeres in S phase. van Overbeek M, de Lange T. Current biology : CB. 2006 16:1295-302. [PubMed:16730176]
18
Recent expansion of the telomeric complex in rodents: Two distinct POT1 proteins protect mouse telomeres. Hockemeyer D, Daniels JP, Takai H, de Lange T. Cell. 2006 126:63-77. [PubMed:16839877]
19
MDC1 accelerates nonhomologous end-joining of dysfunctional telomeres. Dimitrova N, de Lange T. Genes & development. 2006 20:3238-43. [PubMed:17158742] [PMC:PMC1686600]
20
Telomere protection by mammalian Pot1 requires interaction with Tpp1. Hockemeyer D, Palm W, Else T, Daniels JP, Takai KK, Ye JZ, Keegan CE, de Lange T, Hammer GD. Nature structural & molecular biology. 2007 14:754-61. [PubMed:17632522]
21
Cohesin SMC1beta protects telomeres in meiocytes. Adelfalk C, Janschek J, Revenkova E, Blei C, Liebe B, Gb E, Alsheimer M, Benavente R, de Boer E, Novak I, Hg C, Scherthan H, Jessberger R. The Journal of cell biology. 2009 187:185-99. [PubMed:19841137] [PMC:PMC2768837]
22
Metastases suppressor NME2 associates with telomere ends and telomerase and reduces telomerase activity within cells. Kar A, Saha D, Purohit G, Singh A, Kumar P, Yadav VK, Kumar P, Thakur RK, Chowdhury S. Nucleic acids research. 2012 40:2554-65. [PubMed:22135295] [PMC:PMC3315308]
23
Five dysfunctional telomeres predict onset of senescence in human cells. Kaul Z, Cesare AJ, Huschtscha LI, Neumann AA, Reddel RR. EMBO reports. 2012 13:52-9. [PubMed:22157895] [PMC:PMC3246253]
24
A conserved KASH domain protein associates with telomeres, SUN1, and dynactin during mammalian meiosis. Morimoto A, Shibuya H, Zhu X, Kim J, Ishiguro K, Han M, Watanabe Y. The Journal of cell biology. 2012 198:165-72. (ICC; Mouse) [PubMed:22826121] [PMC:PMC3410425]
25
Variant repeats are interspersed throughout the telomeres and recruit nuclear receptors in ALT cells. Conomos D, Stutz MD, Hills M, Neumann AA, Bryan TM, Reddel RR, Pickett HA. The Journal of cell biology. 2012 199:893-906. (ICC; Human) [PubMed:23229897] [PMC:PMC3518223]
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TERF2 / TRF2 Protein - Purified recombinant protein TERF2 was analyzed by SDS-PAGE gel and Coomassie Blue Staining
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TERF2 / TRF2 Protein - Recombinant Telomeric Repeat Binding Factor 2 By SDS-PAGE
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TERF2 / TRF2 Protein - 12.5% SDS-PAGE of human TERF2 stained with Coomassie Blue
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The data on this page has been compiled from LifeSpan internal sources, the National Center for Biotechnology Information (NCBI), and The Universal Protein Resource (UniProt).