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. 2021 May 28;6:36. doi: 10.1038/s41525-021-00198-5

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© The Author(s) 2021

Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/.

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Fig. 2 — Telomeres are noncoding tandem repeats of the sequence TTAGGG, found at the ends of chromosomes in a duplex “D-loop-T-loop” configuration. RTEL1 is a helicase that disrupts T-loops for telomere replication and repair. Shelterin protects telomeres from DNA damage surveillance and comprises of six polypeptide components: TRF1, TRF2, RAP1, TIN2, TPP1 and POT1. Telomerase comprises of the essential components TERT and TERC, which synthesise telomeres and maintain telomere length. Dyskerin forms a complex with NHP2, NOP10 and GAR1. It binds to TERC to stabilise the telomerase complex. TCAB1 regulates the recruitment of telomerase to telomeres. Mutations in any of these components results telomere dysfunction, manifesting as telomere biology disorders. DC, dyskeratosis congenita; GAR1, H/ACA ribonucleoprotein complex subunit 1; HHS, Hoyeraal–Hreidarsson Syndrome; IPF, idiopathic pulmonary fibrosis; NHP2, H/ACA ribonucleoprotein complex subunit 2; NOP10, H/ACA ribonucleoprotein complex subunit 3; POT1, protection of telomeres 1; RAP1, repressor/activator protein 1; RTEL1, regulator of telomere length 1; TCAB1, Telomerase Cajal body protein 1; TERT, telomerase reverse transcriptase; TERC, telomerase RNA component; TIN2, TRF1-interacting nuclear protein 2; TPP1, TIN2-interacting protein 1; TRF1, telomere repeat-binding factor 1; TRF2, telomere repeat-binding factor 2.